Single Ventricle Workup

  • Author: Alvin J Chin, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Nov 10, 2011
 

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),[22] 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.[23]
Next

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-loopCranially 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 echocardioLong 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
    • 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
    • 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.
Previous
Next

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 arrhythmias[24] and conduction block.
    • 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
Previous
Next

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.
Previous
Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

Alvin J Chin, MD  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and International Society for Heart and Lung Transplantation

Disclosure: Actelion Honoraria Speaking and teaching

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.

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.

References

  1. Dhanantwari P, Lee E, Krishnan A, et al. Human cardiac development in the first trimester: a high-resolution magnetic resonance imaging and episcopic fluorescence image capture atlas. Circulation. Jul 28 2009;120(4):343-51. [Medline].

  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. Mar 15 2011;123(10):1125-35. [Medline].

  3. Koshiba-Takeuchi K, Mori AD, Kaynak BL, et al. Reptilian heart development and the molecular basis of cardiac chamber evolution. Nature. Sep 3 2009;461(7260):95-8. [Medline].

  4. Bruneau BG, Logan M, Davis N, Levi T, Tabin CJ, Seidman JG, et al. Chamber-specific cardiac expression of Tbx5 and heart defects in Holt-Oram syndrome. Dev Biol. Jul 1 1999;211(1):100-8. [Medline].

  5. Bruneau BG, Nemer G, Schmitt JP, Charron F, Robitaille L, Caron S. A murine model of Holt-Oram syndrome defines roles of the T-box transcription factor Tbx5 in cardiogenesis and disease. Cell. Sep 21 2001;106(6):709-21. [Medline].

  6. Takeuchi JK, Ohgi M, Koshiba-Takeuchi K, et al. Tbx5 specifies the left/right ventricles and ventricular septum position during cardiogenesis. Development. Dec 2003;130(24):5953-64. [Medline].

  7. Shirakura R, Kawashima Y, Hirose H, Matsuda H, Shimazaki Y, Sano T. Autopsy findings 14 years after septation for single ventricle. Ann Thorac Surg. Jul 1989;48(1):124-5. [Medline].

  8. Fontan F, Mounicot FB, Baudet E, Simonneau J, Gordo J, Gouffrant JM. "Correction" of tricuspid atresia. 2 cases "corrected" using a new surgical technic. Ann Chir Thorac Cardiovasc. Jan 1971;10(1):39-47. [Medline].

  9. Senzaki H, Masutani S, Kobayashi J, et al. Ventricular afterload and ventricular work in fontan circulation: comparison with normal two-ventricle circulation and single-ventricle circulation with blalock-taussig shunts. Circulation. Jun 18 2002;105(24):2885-92. [Medline].

  10. Chin AJ, Whitehead KK, Watrous RL. Insights after 40 years of the Fontan Operation. World Journal of Pediatric and Congenital Heart Surgery. 2010;1:328-343.

  11. de Leval MR, Deanfield JE. Four decades of Fontan Palliation. Nature Reviews Cardiology. 2010;7:520-527.

  12. Cai CL, Liang X, Shi Y, et al. Isl1 identifies a cardiac progenitor population that proliferates prior to differentiation and contributes a majority of cells to the heart. Dev Cell. Dec 2003;5(6):877-89. [Medline].

  13. Lin Q, Schwarz J, Bucana C, Olson EN. Control of mouse cardiac morphogenesis and myogenesis by transcription factor MEF2C. Science. May 30 1997;276(5317):1404-7. [Medline].

  14. Srivastava D, Thomas T, Lin Q, Kirby ML, Brown D, Olson EN. Regulation of cardiac mesodermal and neural crest development by the bHLH transcription factor, dHAND. Nat Genet. Jun 1997;16(2):154-60. [Medline].

  15. Svensson EC, Huggins GS, Lin H, et al. A syndrome of tricuspid atresia in mice with a targeted mutation of the gene encoding Fog-2. Nat Genet. Jul 2000;25(3):353-6. [Medline].

  16. Ilagan R, Abu-Issa R, Brown D, et al. Fgf8 is required for anterior heart field development. Development. Jun 2006;133(12):2435-45. [Medline].

  17. von Both I, Silvestri C, Erdemir T, et al. Foxh1 is essential for development of the anterior heart field. Dev Cell. Sep 2004;7(3):331-45. [Medline].

  18. Sanford LP, Ormsby I, Gittenberger-de Groot AC, et al. TGFbeta2 knockout mice have multiple developmental defects that are non-overlapping with other TGFbeta knockout phenotypes. Development. Jul 1997;124(13):2659-70. [Medline].

  19. Gottlieb PD, Pierce SA, Sims RJ, et al. Bop encodes a muscle-restricted protein containing MYND and SET domains and is essential for cardiac differentiation and morphogenesis. Nat Genet. May 2002;31(1):25-32. [Medline].

  20. Camenisch TD, Spicer AP, Brehm-Gibson T, et al. Disruption of hyaluronan synthase-2 abrogates normal cardiac morphogenesis and hyaluronan-mediated transformation of epithelium to mesenchyme. J Clin Invest. Aug 2000;106(3):349-60. [Medline]. [Full Text].

  21. Zeisberg EM, Ma Q, Juraszek AL, Moses K, Schwartz RJ, Izumo S, et al. Morphogenesis of the right ventricle requires myocardial expression of Gata4. J Clin Invest. Jun 2005;115(6):1522-31. [Medline]. [Full Text].

  22. Narkewicz MR, Sondheimer HM, Ziegler JW, et al. Hepatic dysfunction following the Fontan procedure. J Pediatr Gastroenterol Nutr. Mar 2003;36(3):352-7. [Medline].

  23. Chin AJ, Stephens P, Goldmuntz E, Leonard MB. Serum alkaline phosphatase reflects post-Fontan hemodynamics in children. Pediatr Cardiol. Feb 2009;30(2):138-45. [Medline].

  24. Durongpisitkul K, Porter CJ, Cetta F, et al. Predictors of early- and late-onset supraventricular tachyarrhythmias after Fontan operation. Circulation. Sep 15 1998;98(11):1099-107. [Medline].

  25. Jacobs ML, Rychik J, Rome JJ, et al. Early reduction of the volume work of the single ventricle: the hemi-Fontan operation. Ann Thorac Surg. Aug 1996;62(2):456-61; discussion 461-2. [Medline].

  26. Jacobs ML, Norwood WI Jr. Fontan operation: influence of modifications on morbidity and mortality. Ann Thorac Surg. Oct 1994;58(4):945-51; discussion 951-2. [Medline].

  27. Canobbio MM, Mair DD, van der Velde M, Koos BJ. Pregnancy outcomes after the Fontan repair. J Am Coll Cardiol. Sep 1996;28(3):763-7. [Medline].

  28. Camposilvan S, Milanesi O, Stellin G, Pettenazzo A, Zancan L, D'Antiga L. Liver and cardiac function in the long term after Fontan operation. Ann Thorac Surg. Jul 2008;86(1):177-82. [Medline].

  29. Yeh T Jr, Williams WG, McCrindle BW, et al. Equivalent survival following cavopulmonary shunt: with or without the Fontan procedure. Eur J Cardiothorac Surg. Aug 1999;16(2):111-6. [Medline].

  30. Bromberg BI, Schuessler RB, Gandhi SK, Rodefeld MD, Boineau JP, Huddleston CB. A canine model of atrial flutter following the intra-atrial lateral tunnel Fontan operation. J Electrocardiol. 1998;30 Suppl:85-93. [Medline].

  31. Mavroudis C, Deal BJ, Backer CL, et al. J. Maxwell Chamberlain Memorial Paper for congenital heart surgery. 111 Fontan conversions with arrhythmia surgery: surgical lessons and outcomes. Ann Thorac Surg. Nov 2007;84(5):1457-65; discussion 1465-6. [Medline].

  32. Petrossian E, Reddy VM, McElhinney DB, et al. Early results of the extracardiac conduit Fontan operation. J Thorac Cardiovasc Surg. Apr 1999;117(4):688-96. [Medline].

  33. Kiesewetter CH, Sheron N, Vettukattill JJ, et al. Hepatic changes in the failing Fontan circulation. Heart. May 2007;93(5):579-84. [Medline]. [Full Text].

  34. Wilson WR, Greer GE, Tobias JD. Cerebral venous thrombosis after the Fontan procedure. J Thorac Cardiovasc Surg. Oct 1998;116(4):661-3. [Medline].

  35. Barber BJ, Burch GH, Tripple D, Balaji S. Resolution of plastic bronchitis with atrial pacing in a patient with fontan physiology. Pediatr Cardiol. Jan-Feb 2004;25(1):73-6. [Medline].

  36. Fujii T, Shimizu T, Takahashi K, et al. Fecal alpha1-antitrypsin concentrations as a measure of enteric protein loss after modified fontan operations. J Pediatr Gastroenterol Nutr. Nov 2003;37(5):577-80. [Medline].

  37. Cheung YF, Tsang HY, Kwok JS. Immunologic profile of patients with protein-losing enteropathy complicating congenital heart disease. Pediatr Cardiol. Nov-Dec 2002;23(6):587-93. [Medline].

  38. Pekkan K, Kitajima HD, de Zelicourt D, et al. Total cavopulmonary connection flow with functional left pulmonary artery stenosis: angioplasty and fenestration in vitro. Circulation. Nov 22 2005;112(21):3264-71. [Medline].

  39. Jacobs ML, Schneider DJ, Pourmoghadam KK, Pizarro C, Norwood WI. Total cavopulmonary connection to one lung. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:72-9. [Medline].

  40. Troutman WB, Barstow TJ, Galindo AJ, Cooper DM. Abnormal dynamic cardiorespiratory responses to exercise in pediatric patients after Fontan procedure. J Am Coll Cardiol. Mar 1 1998;31(3):668-73. [Medline].

  41. Chang RK, Alejos JC, Atkinson D, Jensen R, Drant S, Galindo A, et al. Bubble contrast echocardiography in detecting pulmonary arteriovenous shunting in children with univentricular heart after cavopulmonary anastomosis. J Am Coll Cardiol. Jun 1999;33(7):2052-8. [Medline].

  42. Barber BJ, Burch GH, Tripple D, Balaji S. Resolution of plastic bronchitis with atrial pacing in a patient with fontan physiology. Pediatr Cardiol. Jan-Feb 2004;25(1):73-6. [Medline].

  43. Wilson J, Russell J, Williams W, Benson L. Fenestration of the Fontan circuit as treatment for plastic bronchitis. Pediatr Cardiol. Sep-Oct 2005;26(5):717-9. [Medline].

  44. Semenza GL. Expression of hypoxia-inducible factor 1: mechanisms and consequences. Biochem Pharmacol. Jan 1 2000;59(1):47-53. [Medline].

  45. Deal BJ, Backer CL, Ward KM, Tsao S, Dechert B, de Freitas A, et al. Medical characteristics and mid-term outcome: 100 consecutive Fontan conversions with arrhythmia surgery. Circulation. 2007;116:II-414.

  46. Kaushansky K. Lineage-specific hematopoietic growth factors. N Engl J Med. May 11 2006;354(19):2034-45. [Medline].

  47. Rydberg A, Teien DE, Krus P. Computer simulation of circulation in patient with total cavo-pulmonary connection: inter-relationship of cardiac and vascular pressure, flow, resistance and capacitance. Med Biol Eng Comput. Nov 1997;35(6):722-8. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.