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Single Ventricle

Author: Joseph Heaton Author: Mohamed H. Alahmadi Editor: Daniel Heller Updated: 12/11/2024 10:33:23 PM

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

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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—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 anomalies, 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 use is associated with an increased risk of cardiac malformations, specifically at higher doses and when used during the 1st trimester [10][11]

 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.

Epidemiology

The occurrence of congenital heart disease is between 6 and 13 in 1,000 live births.[12][13][14] HLHS, the most common form of univentricular heart disease, is seen in 2 to 3 per 10,000 births, with a higher incidence in male infants.[15][16][17] Tricuspid atresia occurs in about 1 per 10,000 live births.[18][19] Ebstein anomaly is observed in about 0.5 per 10,000 live births with sex predilection.[20] However, with maternal use of lithium, the likelihood of Ebstein anomaly can increase nearly 7 times.[21] Double outlet right ventricle occurs in 0.009 cases per 10,000 live births.[22] Double inlet left ventricle is reported in up to 0.01 per 10,000 live births.[23] Atrioventricular canal defect is found in 0.03 to 0.04 per 10,000 live births.[24]

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 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, over time, usually within 25 years, half of the patients who undergo the procedure are expected to experience Fontan failure, a condition marked by circulatory dysfunction, reduced functional capacity (New York Heart Association [NYHA] class III or IV), Fontan takedown or conversion, severe complications such as protein-losing enteropathy (PLE) and plastic bronchitis, the need for heart transplantation, or mortality.[25][26]

History and Physical

A single ventricle can be identified as early as the 18th week of gestation.[27] 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.[28][29]

Postnatal presentations vary based on underlying structural abnormalities. Typically, signs such as a heart murmur, tachypnea, respiratory distress, cyanosis, or hypotension may occur when circulation and oxygenation are inadequate.[30][31] Additional physical examination findings, like hepatomegaly or dysmorphic features, may indicate other underlying issues. 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 computed tomography, cardiac catheterization, magnetic resonance imaging, or cardiac magnetic resonance imaging are typically reserved for complex cases requiring further evaluation or treatment planning.

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.

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.[34] Inhaled nitric oxide may reduce pulmonary vascular resistance, improving oxygenation by enhancing pulmonary blood flow.[35] 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.[36] 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.[37][38][39] 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.[40](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.[41] Optimal pressure dynamics and low pulmonary vascular resistance are critical for maintaining effective anterograde circulation.[42] 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.[43] 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.[44] Cardiac transplantation may be considered, though it is associated with suboptimal outcomes due to comorbidities.[45][46] 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 collateral circulations diminish. 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.[47] 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, but the survival rate improves to 60% to 70% with partial surgical revisions.[48][49][50] Patients who survive beyond a year after surgical intervention have a 90% likelihood of reaching 18 years of age.[51] Despite improved survival rates, the risk of suboptimal neurodevelopment may be increased, regardless of the intervention performed.[52][53] The American Heart Association recommends developmental evaluation for these patients, as neurodevelopmental issues may result from inadequate nutritional delivery.[54][55]

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] Ebstein anomaly, however, 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 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
    • Male sex [61]
    • Common atrioventricular valve [62]
    • HLHS, although newer studies have found a weaker correlation between worse outcomes and individuals with this condition who have undergone the Fontan procedure [63][64]
    • Mean pulmonary artery pressure greater than 16 mm Hg
  • Intraoperative factors
    • Atriopulmonary Fontan [65]
    • Lack of septal fenestrations [66]
    • Late intervention [67]
    • Complicated procedure, eg, prolonged cross-clamp time [68]
  • Postoperative factors
    • Early:
      • Fontan circulation pressure greater than 20 mm Hg
      • Unsettled chest drainage lasting longer than 3 weeks [69]
      • Ventricular filling pressure greater than 13 mm Hg
    • Late:
      • PLE [70]
      • Plastic bronchitis [71]
      • Reduced exercise capacity, as signified by a decreased peak VO2 [72]
      • Arrhythmias [73]
      • Need for pacing [74]

Complications

Single-ventricle heart syndromes are typically corrected through direct intervention, obfuscating the origin of certain complications. The expected sequelae of corrected single-ventricle hearts are listed below.

  • Arrhythmias [75][76]
  • Esophageal varices [77]
  • Heart failure with thrombus formation and increased risk of bleeding from treatment [78][79]
  • Increased risk for decompensation with anesthesia [80]
  • Long-term cyanosis [81]
  • Restrictive and diffusion-limited lung disease.[82][83] Pulmonary disease in these patients may be partly due to subclinical plastic bronchitis and subclinical pulmonary embolisms.[84]
  • PLE [85][86]
  • Recurrent laryngeal nerve injury [87]
  • Reduced height and somatic development [88]
  • Renal dysfunction
  • Systemic venous-to-pulmonary venous and systemic artery-to-pulmonary artery collaterals

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 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 considered as part of the discussion of available options. Additionally, living conditions should be addressed with patients, particularly for families living at altitudes higher than 1,700 meters above sea level, as residing in such locations may negatively impact long-term survival.[89]

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

[1]

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]

[2]

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) evidence

[3]

Kennedy 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) evidence

[4]

Shapiro 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]

[5]

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) evidence

[6]

Zeisberg 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) evidence

[7]

Towbin 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) evidence

[8]

Materna-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]

[9]

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]

[10]

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]

[11]

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) evidence

[12]

Khoshnood B, Lelong N, Houyel L, Thieulin AC, Jouannic JM, Magnier S, Delezoide AL, Magny JF, Rambaud C, Bonnet D, Goffinet F, EPICARD Study Group. Prevalence, timing of diagnosis and mortality of newborns with congenital heart defects: a population-based study. Heart (British Cardiac Society). 2012 Nov:98(22):1667-73. doi: 10.1136/heartjnl-2012-302543. Epub 2012 Aug 11     [PubMed PMID: 22888161]

Level 2 (mid-level) evidence

[13]

Liu S, Joseph KS, Lisonkova S, Rouleau J, Van den Hof M, Sauve R, Kramer MS, Canadian Perinatal Surveillance System (Public Health Agency of Canada). Association between maternal chronic conditions and congenital heart defects: a population-based cohort study. Circulation. 2013 Aug 6:128(6):583-9. doi: 10.1161/CIRCULATIONAHA.112.001054. Epub 2013 Jun 28     [PubMed PMID: 23812182]

Level 2 (mid-level) evidence

[14]

Ferencz C, Rubin JD, McCarter RJ, Brenner JI, Neill CA, Perry LW, Hepner SI, Downing JW. Congenital heart disease: prevalence at livebirth. The Baltimore-Washington Infant Study. American journal of epidemiology. 1985 Jan:121(1):31-6     [PubMed PMID: 3964990]

Level 2 (mid-level) evidence

[15]

Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. The Journal of pediatrics. 2008 Dec:153(6):807-13. doi: 10.1016/j.jpeds.2008.05.059. Epub 2008 Jul 26     [PubMed PMID: 18657826]

[16]

Fixler DE, Nembhard WN, Salemi JL, Ethen MK, Canfield MA. Mortality in first 5 years in infants with functional single ventricle born in Texas, 1996 to 2003. Circulation. 2010 Feb 9:121(5):644-50. doi: 10.1161/CIRCULATIONAHA.109.881904. Epub 2010 Jan 25     [PubMed PMID: 20100974]

Level 2 (mid-level) evidence

[17]

Karamlou T, Diggs BS, Ungerleider RM, Welke KF. Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period. The Journal of thoracic and cardiovascular surgery. 2010 Jan:139(1):119-26; discussion 126-7. doi: 10.1016/j.jtcvs.2009.04.061. Epub 2009 Nov 11     [PubMed PMID: 19909991]

[18]

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]

[19]

Rao PS. Tricuspid Atresia. Current treatment options in cardiovascular medicine. 2000 Dec:2(6):507-520     [PubMed PMID: 11096554]

[20]

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]

[21]

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) evidence

[22]

Demir 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) evidence

[23]

Gidvani 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) evidence

[24]

Hoffman JI. Incidence of congenital heart disease: I. Postnatal incidence. Pediatric cardiology. 1995 May-Jun:16(3):103-13     [PubMed PMID: 7617503]

[25]

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]

[26]

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]

[27]

Tongsong T, Sittiwangkul R, Wanapirak C, Chanprapaph P. Prenatal diagnosis of isolated tricuspid valve atresia: report of 4 cases and review of the literature. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. 2004 Jul:23(7):945-50     [PubMed PMID: 15292563]

Level 3 (low-level) evidence

[28]

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) evidence

[29]

Galindo 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]

[30]

Forbess JM, Cook N, Roth SJ, Serraf A, Mayer JE Jr, Jonas RA. Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality. Circulation. 1995 Nov 1:92(9 Suppl):II262-6     [PubMed PMID: 7586421]

[31]

Vlahos AP, Lock JE, McElhinney DB, van der Velde ME. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy. Circulation. 2004 May 18:109(19):2326-30     [PubMed PMID: 15136496]

Level 2 (mid-level) evidence

[32]

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) evidence

[33]

Monaco 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) evidence

[34]

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]

[35]

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]

[36]

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) evidence

[37]

Mäkikallio 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) evidence

[38]

Tworetzky 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) evidence

[39]

Freud 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]

[40]

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) evidence

[41]

Shafer 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]

[42]

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]

[43]

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]

[44]

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) evidence

[45]

Alsoufi 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) evidence

[46]

Alsoufi 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]

[47]

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) evidence

[48]

Newburger JW, Sleeper LA, Gaynor JW, Hollenbeck-Pringle D, Frommelt PC, Li JS, Mahle WT, Williams IA, Atz AM, Burns KM, Chen S, Cnota J, Dunbar-Masterson C, Ghanayem NS, Goldberg CS, Jacobs JP, Lewis AB, Mital S, Pizarro C, Eckhauser A, Stark P, Ohye RG, Pediatric Heart Network Investigators. Transplant-Free Survival and Interventions at 6 Years in the SVR Trial. Circulation. 2018 May 22:137(21):2246-2253. doi: 10.1161/CIRCULATIONAHA.117.029375. Epub 2018 Feb 1     [PubMed PMID: 29437119]

[49]

Scheurer MA, Salvin JW, Vida VL, Fynn-Thompson F, Bacha EA, Pigula FA, Mayer JE Jr, del Nido PJ, Wessel DL, Laussen PC, Thiagarajan RR. Survival and clinical course at Fontan after stage one palliation with either a modified Blalock-Taussig shunt or a right ventricle to pulmonary artery conduit. Journal of the American College of Cardiology. 2008 Jul 1:52(1):52-9. doi: 10.1016/j.jacc.2008.03.034. Epub     [PubMed PMID: 18582635]

Level 2 (mid-level) evidence

[50]

d'Udekem Y, Xu MY, Galati JC, Lu S, Iyengar AJ, Konstantinov IE, Wheaton GR, Ramsay JM, Grigg LE, Millar J, Cheung MM, Brizard CP. Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance. Journal of the American College of Cardiology. 2012 Mar 27:59(13):1178-85. doi: 10.1016/j.jacc.2011.11.049. Epub     [PubMed PMID: 22440217]

Level 2 (mid-level) evidence

[51]

Siffel C, Riehle-Colarusso T, Oster ME, Correa A. Survival of Children With Hypoplastic Left Heart Syndrome. Pediatrics. 2015 Oct:136(4):e864-70. doi: 10.1542/peds.2014-1427. Epub 2015 Sep 21     [PubMed PMID: 26391936]

[52]

Laraja K, Sadhwani A, Tworetzky W, Marshall AC, Gauvreau K, Freud L, Hass C, Dunbar-Masterson C, Ware J, Lafranchi T, Wilkins-Haug L, Newburger JW. Neurodevelopmental Outcome in Children after Fetal Cardiac Intervention for Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome. The Journal of pediatrics. 2017 May:184():130-136.e4. doi: 10.1016/j.jpeds.2017.01.034. Epub 2017 Feb 21     [PubMed PMID: 28233547]

[53]

Goldberg CS, Lu M, Sleeper LA, Mahle WT, Gaynor JW, Williams IA, Mussatto KA, Ohye RG, Graham EM, Frank DU, Jacobs JP, Krawczeski C, Lambert L, Lewis A, Pemberton VL, Sananes R, Sood E, Wechsler SB, Bellinger DC, Newburger JW, Pediatric Heart Network Investigators. Factors associated with neurodevelopment for children with single ventricle lesions. The Journal of pediatrics. 2014 Sep:165(3):490-496.e8. doi: 10.1016/j.jpeds.2014.05.019. Epub 2014 Jun 19     [PubMed PMID: 24952712]

Level 1 (high-level) evidence

[54]

Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, Mussatto KA, Uzark K, Goldberg CS, Johnson WH Jr, Li J, Smith SE, Bellinger DC, Mahle WT, American Heart Association Congenital Heart Defects Committee, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, and Stroke Council. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012 Aug 28:126(9):1143-72     [PubMed PMID: 22851541]

[55]

Ravishankar C, Zak V, Williams IA, Bellinger DC, Gaynor JW, Ghanayem NS, Krawczeski CD, Licht DJ, Mahony L, Newburger JW, Pemberton VL, Williams RV, Sananes R, Cook AL, Atz T, Khaikin S, Hsu DT, Pediatric Heart Network Investigators. Association of impaired linear growth and worse neurodevelopmental outcome in infants with single ventricle physiology: a report from the pediatric heart network infant single ventricle trial. The Journal of pediatrics. 2013 Feb:162(2):250-6.e2. doi: 10.1016/j.jpeds.2012.07.048. Epub 2012 Aug 30     [PubMed PMID: 22939929]

Level 1 (high-level) evidence

[56]

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]

[57]

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) evidence

[58]

Freud 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) evidence

[59]

Celermajer 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) evidence

[60]

Yeh 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) evidence

[61]

Burkhart 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]

[62]

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]

[63]

Iyengar AJ, Winlaw DS, Galati JC, Wheaton GR, Gentles TL, Grigg LE, Justo RN, Radford DJ, Weintraub RG, Bullock A, Celermajer DS, d'Udekem Y, Australia and New Zealand Fontan Registry. The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 2014 Sep:46(3):465-73; discussion 473. doi: 10.1093/ejcts/ezu015. Epub 2014 Feb 26     [PubMed PMID: 24578409]

[64]

Martin BJ, Mah K, Eckersley L, Harder J, Pockett C, Schantz D, Dyck J, Al Aklabi M, Rebeyka IM, Ross DB. Hypoplastic Left Heart Syndrome Is Not a Predictor of Worse Intermediate Mortality Post Fontan. The Annals of thoracic surgery. 2017 Dec:104(6):2037-2044. doi: 10.1016/j.athoracsur.2017.08.032. Epub 2017 Oct 31     [PubMed PMID: 29096870]

[65]

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) evidence

[66]

Lemler MS, Scott WA, Leonard SR, Stromberg D, Ramaciotti C. Fenestration improves clinical outcome of the fontan procedure: a prospective, randomized study. Circulation. 2002 Jan 15:105(2):207-12     [PubMed PMID: 11790702]

Level 1 (high-level) evidence

[67]

Fontan F, Kirklin JW, Fernandez G, Costa F, Naftel DC, Tritto F, Blackstone EH. Outcome after a "perfect" Fontan operation. Circulation. 1990 May:81(5):1520-36     [PubMed PMID: 2331765]

[68]

Mitchell 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]

[69]

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]

[70]

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]

[71]

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]

[72]

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]

[73]

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]

[74]

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]

[75]

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) evidence

[76]

Durongpisitkul 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]

[77]

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]

[78]

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]

[79]

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]

[80]

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) evidence

[81]

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]

[82]

Matthews 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) evidence

[83]

Idorn 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) evidence

[84]

Liptzin 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]

[85]

Rychik 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]

[86]

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]

[87]

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]

[88]

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) evidence

[89]

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

[90]

Dasí F. Alpha-1 antitrypsin deficiency. Medicina clinica. 2024 Apr 12:162(7):336-342. doi: 10.1016/j.medcli.2023.10.014. Epub 2023 Nov 22     [PubMed PMID: 37993348]