eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Single Ventricle: Differential Diagnoses & Workup

Author: Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Contributor Information and Disclosures

Updated: Jul 15, 2009

Differential Diagnoses

Aortic Stenosis, Valvar
Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure
Corrected Transposition of the Great Arteries: Surgical Perspective
Neonatal Sepsis
Double Outlet Right Ventricle: Surgical Perspective
Protein-Losing Enteropathy
Heterotaxy, Asplenia
Pulmonary Stenosis, Valvar
Heterotaxy, Polysplenia
Hypoplastic Left Heart Syndrome

Other Problems to Be Considered

  • Pulmonary stenosis or complex heart malformation with pulmonary stenosis as a component
  • Arch obstruction or complex heart malformation with aortic stenosis, arch obstruction, or both as a component
  • Neonatal sepsis

Workup

Laboratory Studies

  • No specific laboratory blood tests are required in the preoperative workup for single ventricle.
  • An ABG measurement is frequently helpful in distinguishing between cases of single ventricle with subpulmonary stenosis and those cases of single ventricle with arch obstruction, aortic stenosis, or both. For example, when prostaglandin E1 has not been administered, a PaO2 of greater than 50 mm Hg lessens the likelihood that a newborn with single ventricle has significant subpulmonary stenosis. However, this PaO2 is perfectly consistent with the presence of arch obstruction.
  • Following Fontan operation, albumin and total protein levels are helpful in surveillance for protein-losing enteropathy, which is a known complication. Fecal alpha1-antitrypsin helps prove that the hypoproteinemia is at least partly due to excessive intestinal loss. Prolongation in the prothrombin time (a measure of hepatic synthetic function),16 abnormally elevated gammaglutamyltranspeptidase, and a reduction in alkaline phosphatase levels (largely a reflection of osteoblastic activity in preadolescent children) are likely early clues to hepatic dysfunction, biliary dysfunction, and reduced bone formation, respectively.17

Imaging Studies

  • Two-dimensional echocardiography and Doppler analysis

    Pulsed Doppler echocardiographic evaluation of th...

    Pulsed Doppler echocardiographic evaluation of the narrowing shown in Media file 2. On the left, the sample volume (split oval) is placed just distal to the narrow area. On the right, spectral analysis reveals a mixture of velocities, consistent with the presence of highly nonlaminar (turbulent) flow.

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    Pulsed Doppler echocardiographic evaluation of th...

    Pulsed Doppler echocardiographic evaluation of the narrowing shown in Media file 2. On the left, the sample volume (split oval) is placed just distal to the narrow area. On the right, spectral analysis reveals a mixture of velocities, consistent with the presence of highly nonlaminar (turbulent) flow.

    • Two-dimensional echocardiography is diagnostic for single ventricle. The presence or absence of subpulmonary stenosis, arch obstruction, and aortic stenosis can also be determined. The particular atrioventricular connection and ventriculoarterial alignment is also revealed in a straightforward manner.
    • The 2 most common forms of single ventricle are L-looped single left ventricle (LV) with transposition of the great arteries and subpulmonary stenosis and D-looped single LV with transposition of the great arteries and subpulmonary stenosis. The third most common form is L-looped single LV with transposition of the great arteries and aortic arch hypoplasia.

      Cranially angulated frontal angiogram of an L-loo...

      Cranially angulated frontal angiogram of an L-looped single left ventricle. Abbreviations are as follows: ao=aorta, mpa=main pulmonary artery, oc=outlet chamber (rudimentary right ventricle).

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      Cranially angulated frontal angiogram of an L-loo...

      Cranially angulated frontal angiogram of an L-looped single left ventricle. Abbreviations are as follows: ao=aorta, mpa=main pulmonary artery, oc=outlet chamber (rudimentary right ventricle).

    • In single LV with transposition of the great arteries and aortic arch obstruction, the aortic stenosis that frequently coexists is due to a narrowing at the communication between the LV and the rudimentary right ventricle (RV). This orifice is frequently referred to as a bulboventricular foramen or outlet foramen.
  • Echocardiography prior to initial surgery

    Long axial oblique-equivalent subcostal echocardi...

    Long axial oblique-equivalent subcostal echocardiogram of single left ventricle (vent) with narrow communication (unlabeled arrow) between left ventricle and outlet chamber (oc). Abbreviations are as follows: L=left, lav=left atrioventricular valve, P=posterior, rav=right atrioventricular valve, S=superior.

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    Long axial oblique-equivalent subcostal echocardi...

    Long axial oblique-equivalent subcostal echocardiogram of single left ventricle (vent) with narrow communication (unlabeled arrow) between left ventricle and outlet chamber (oc). Abbreviations are as follows: L=left, lav=left atrioventricular valve, P=posterior, rav=right atrioventricular valve, S=superior.

    • Initial identification of single ventricle
    • Presence or absence of subpulmonary stenosis
    • Presence or absence of arch obstruction
    • Presence or absence of narrowing of communication between normal-sized ventricle and rudimentary ventricle
    • Presence or absence of atrioventricular valve regurgitation, which would have to be palliated prior to Fontan operation
    • Presence or absence of pulmonary artery distortion
    • Ventricular performance
  • Echocardiography prior to hemi-Fontan (or bidirectional Glenn) operation
    • Presence or absence of pulmonary artery distortion, either congenital or created inadvertently by prior pulmonary artery surgery
    • Presence or absence of second superior vena cava
    • Ventricular performance
  • Echocardiography prior to Fontan operation (whether lateral tunnel or extracardiac conduit)
    • Determination of whether a pulmonary arteriovenous malformation has developed
    • Presence or absence of pulmonary artery distortion, either congenital or created inadvertently by prior pulmonary artery surgery 
    • Ventricular performance
  • Echocardiography after Fontan operation
    • Characterization of the fenestration, in cases in which a fenestrated Fontan solution is adopted
    • Presence or absence of effusions, pericardial or pleural, or ascites
    • Presence or absence of thrombus formation
    • Presence or absence of pulmonary venous pathway obstruction
    • Ventricular performance
  • Chest radiography
    • Chest radiography findings vary.
    • In cases with pulmonary stenosis, the cardiac silhouette is normal to mildly enlarged. Pulmonary vascularity is not increased.
    • In cases with arch obstruction, the cardiac silhouette is usually at least mildly enlarged. Pulmonary vascularity usually is increased.

Other Tests

  • Electrocardiography: Common findings include septal q wave in the right precordial leads (in cases of L-looped single LV) and a monotonous R/S pattern over the anterior precordium.
  • Holter monitoring: This is useful after a hemi-Fontan operation (or bidirectional Glenn operation) and is particularly helpful after a Fontan operation for surveillance of supraventricular arrhythmias18 and conduction block.
  • Cardiac MRI
    • Anatomy - Static, steady-state free precession (SSFP) bright blood images; double-inversion, dark blood images; half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences 
    • Physiology - Stack of cines (short axis of ventricle, to analyze ventricular performance), cines of systemic venous pathway and pulmonary arteries 
    • Velocity mapping of superior vena cava, inferior vena cava, branch pulmonary arteries, and aorta
    • Post–gadolinium injection, 3-dimensional reconstruction, and viability imaging

Procedures

  • Cardiac catheterization is largely reserved for evaluating candidacy for Fontan operation, characterizing post-Fontan hemodynamics, and managing supraventricular arrhythmic complications.
  • Postcatheterization precautions include hemorrhage, vascular disruption after balloon dilation, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm.
  • Complications may include rupture of blood vessel, tachyarrhythmias, bradyarrhythmias, and vascular occlusion.

More on Single Ventricle

Overview: Single Ventricle
Differential Diagnoses & Workup: Single Ventricle
Treatment & Medication: Single Ventricle
Follow-up: Single Ventricle
Multimedia: Single Ventricle
References
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Further Reading

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Keywords

single ventricle, single left ventricle, single right ventricle, tricuspid atresia, mitral atresia, double-inlet single ventricle, common-inlet single ventricle, hepatic dysfunction, biliary dysfunction, protein-losing enteropathy, subpulmonary stenosis, aortic arch obstruction, aortic arch hypoplasia, subaortic stenosis, cyanosis, peripheral perfusion, Fontan operation, hypoproteinemia, L-looped single left ventricle, transposition of the great arteries, D-looped single left ventricle, bulboventricular foramen, outlet foramen, bidirectional Glenn operation, hemi-Fontan operation, pulmonary artery distortion, pericardial effusion, pleural effusion, ascites, thrombus, sinus bradycardia, atrial tachyarrhythmias, varices, plastic bronchitis, thrombocytopenia, systemic collaterals, short stature, treatment, diagnosis

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Contributor Information and Disclosures

Author

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation
Disclosure: Actelion Honoraria Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Ameeta Martin, MD, Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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