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Single Ventricle Workup

  • Author: Alvin J Chin, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Jan 05, 2016
 

Laboratory Studies

No specific laboratory blood tests are required in the preoperative workup for single ventricle, although, in the near future, affordable whole-genome sequencing will likely be helpful.

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, fecal alpha1-antitrypsin measurement is crucial in surveillance for the complication of protein-losing enteropathy (PLE). Abnormalities in serum total protein and albumin are relatively late clues to PLE; because the liver is the sole site of endogenous albumin production, a low serum albumin level signifies the liver's inability to compensate for poor protein intake or excessive protein loss. Prolongation in the prothrombin time (a measure of hepatic synthetic function),[48] 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.[30]

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Imaging Studies

Two-dimensional echocardiography and Doppler analysis

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 (see the image below) 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. The fourth most common form is D-looped single LV with normally aligned great arteries (ie, aorta from LV and pulmonary artery from outlet chamber), which is sometimes referred to as a Holmes heart.

Cranially angulated frontal angiogram of an L-loop 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 (sub)aortic stenosis that frequently coexists is due to a narrowing at the communication between the LV and the rudimentary right ventricle (outlet chamber). See the image below. This orifice is frequently referred to as a bulboventricular foramen or outlet foramen.

Long axial oblique-equivalent subcostal echocardio 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.

Echocardiography prior to initial surgery

This study is used for evaluation of the following:

  • 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 straddling AV valve (ie, the AV valve closer to the outlet chamber having attachments to the rim of the outlet foramen or actually within the outlet chamber): The presence of such attachments should be an absolute contraindication to surgical enlargement of the outlet foramen which might otherwise be contemplated in cases of late-onset "subaortic stenosis."
  • 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

This study is used for evaluation of the following:

  • 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

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.

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 arrhythmias[49] and conduction block.

This study is used for evaluation of the following:

  • 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
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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.

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Contributor Information and Disclosures
Author

Alvin J Chin, MD Emeritus Professor of Pediatrics, 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, Society for Developmental Biology, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

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, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

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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).
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.
Cardiac MRI. Frontal view of a 3-dimensional flow field in a patient who has undergone a lateral tunnel type of modified Fontan operation (A). This surgical palliation for patients with only one functional ventricle redirects venous blood from the superior vena cava (SVC) and inferior vena cava (IVC) directly into the right (RPA) and left (LPA) pulmonary arteries. Flow streamlines are shown in red. B. Frontal view of in plane velocity mapping. Right (R jug) and left (L jug) jugular vein flow towards the feet is signal-poor (black). Flow toward the head in the infrahepatic inferior vena cava (IVC) and intracardiac portion of the systemic venous pathway (svp) is signal-intense (white). Images courtesy of Dr. Mark A. Fogel, The Children's Hospital of Philadelphia.
A sharp left-right gradient in Tbx5 expression is required for the formation of the ventricular septum. Image from Zina Deretsky, National Science Foundation after Benoit Brueau, the Gladstone Institute of Cardiovascular Disease.
 
 
 
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