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Muscular Ventricular Septal Defect Treatment & Management

  • Author: Michael D Taylor, MD, PhD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Jan 04, 2016
 

Approach Considerations

Small muscular ventricular septal defects (VSDs) have a high spontaneous closure rate (80-90%) within the first 2 years of life and often require no medical or surgical management. Larger defects may not close but may become smaller with time.

Medical therapy may be required with large muscular VSDs due to excessive left-to-right shunting and the development of CHF. Therapy is directed at alleviating the symptoms of pulmonary overcirculation and typically includes increased-calorie feedings, diuretics, and, sometimes, an ACE inhibitor.

Diuretic therapy with furosemide is used to lessen volume overload. Significant potassium wasting may warrant the addition of spironolactone or potassium supplementation.

The use of afterload reduction to improve systemic-pulmonary flow ratios may be beneficial in selected cases. ACE inhibitors also inhibit the tissue-based renin-angiotensin system, preventing deleterious remodeling. Be aware that ACE inhibitors have a potassium-sparing effect. When these are used, spironolactone or supplemental potassium should be avoided or judiciously used.

Surgical indications

Failure of medical management to alleviate symptoms in the first 6 months of life requires intervention. Growth failure despite optimal medical therapy and maximized calorie intake is the most important evidence of failure of medical therapy. Intervention is either by surgery or by cardiac catheterization. Very large left-to-right shunts are usually electively repaired within the first year of life.

Elevated pulmonary arteriolar resistance of more than 12 Wood units that does not decrease with oxygen or selective pulmonary vasodilator therapy may be regarded as inoperable.

Diet

In patients with small muscular VSDs, no special diet is required. For patients with large muscular VSDs and significant CHF, caloric supplementation is often required using fortified formula or breast milk.

Activity

Patients with small muscular VSDs can maintain normal activity. Patients with moderate to large defects with significant symptomatology may self-limit strenuous exercise until the defect is repaired. Patients with repaired VSD and no residual cardiac sequelae can resume regular activity.

Inpatient care

Routine inpatient monitoring of infants and children with a small muscular VSD is not necessary. Hospitalization for severe CHF usually indicates the need for early intervention for VSD closure.

Transfer

Transfer to a tertiary care center may be required for further diagnostic evaluation or surgical intervention in patients with large VSDs or multiple VSDs.

Consultations

Consultations may be indicated with the following specialists:

  • Pediatric cardiologist
  • Pediatric cardiothoracic surgeon
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Surgical Treatment

Surgical repair is the most common intervention currently performed.[11] New surgical approaches using smaller incisions have proven effective in single ventricular septal defect (VSD) closure.[12] Surgical repair of an isolated large VSD involves closure of the muscular defect with a Gore-Tex patch.

Surgical intervention in younger infants, especially those younger than 1 month, is associated with an increased risk of mortality (historically as high as 10-20%, although currently much lower). Surgical mortality is now very low (approximately 1%) in patients older than 6 months with isolated large muscular VSDs.

Patients with multiple muscular VSDs may undergo pulmonary artery banding if primary repair is deemed too risky. This palliative procedure limits the degree of left-to-right shunting and allows additional time for these defects to decrease in size or undergo spontaneous closure.

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Cardiac Catheterization and Hybrid Procedures

Devices are now available for closure of muscular ventricular septal defects (VSDs).[13] VSD closure devices typically have 2 opposing discs (one for the right ventricular side and one for the left ventricular side), which are released during catheterization under fluoroscopic and transesophageal echocardiographic guidance to occlude the defect. Data suggest that closure of small muscular VSDs in patients with otherwise normal anatomy offers less short-term morbidity with similar results. However, the long-term morbidity is unknown.[14, 15, 16, 17, 18, 19]

The closure devices can be percutaneously placed in the cardiac catheterization laboratory or in the operating room during a "hybrid procedure."[20, 21, 22] Hybrid procedures may involve inserting the device through a very small incision in the free wall of the right ventricle.

Ongoing investigational trials are currently being performed to assess indications and outcomes in VSD closure with these devices. Data suggest that the ventricular septal occluder does not cause interventricular conduction disturbances in greater numbers than does surgical repair.

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Outpatient Care

Mild to moderate congestive heart failure (CHF) secondary to left-to-right shunting from a ventricular septal defect (VSD) may be managed in an outpatient setting. For routine muscular VSDs, prophylactic antibiotics for the prevention of bacterial endocarditis are no longer recommended by the American Heart Association, except under special circumstances.[23] A modest risk of endocarditis is still observed; thus, the importance of vigilant oral hygiene should be reinforced.

Small muscular VSDs have a high incidence of spontaneous closure. Serial follow-up care should be performed until spontaneous closure occurs. Moderate-sized VSDs can also be followed in the outpatient setting while awaiting evidence of reduction in size or spontaneous closure. Patients with a large VSD but without significant CHF can be followed as outpatients, with frequent evaluations.

Serial patient follow-up care is required for assessment of patient growth and ongoing evaluation of the need for elective surgical closure.

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Contributor Information and Disclosures
Author

Michael D Taylor, MD, PhD Director, Advanced Imaging Innovation, Cincinnati Children's Hospital Medical Center; Assistant Professor, Department of Pediatrics, University of Cincinnati College of Medicine

Michael D Taylor, MD, PhD is a member of the following medical societies: American College of Cardiology, American Heart Association, Society for Cardiovascular Magnetic Resonance

Disclosure: Nothing to disclose.

Coauthor(s)

Benjamin W Eidem, MD, FACC, FASE Professor of Pediatrics and Medicine, Departments of Pediatrics and Medicine, Divisions of Pediatric Cardiology and Cardiovascular Diseases, Mayo Medical School

Benjamin W Eidem, MD, FACC, FASE is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Acknowledgements

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

Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

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.

References
  1. Ramaciotti C, Vetter JM, Bornemeier RA, Chin AJ. Prevalence, relation to spontaneous closure, and association of muscular ventricular septal defects with other cardiac defects. Am J Cardiol. 1995 Jan 1. 75(1):61-5. [Medline].

  2. Newman TB. Etiology of ventricular septal defects: an epidemiologic approach. Pediatrics. 1985 Nov. 76(5):741-9. [Medline].

  3. Nicolae MI, Summers KM, Radford DJ. Familial muscular ventricular septal defects and aneurysms of the muscular interventricular septum. Cardiol Young. 2007 Oct. 17(5):523-7. [Medline].

  4. Williams LJ, Correa A, Rasmussen S. Maternal lifestyle factors and risk for ventricular septal defects. Birth Defects Res A Clin Mol Teratol. 2004 Feb. 70(2):59-64. [Medline].

  5. Oberlander TF, Warburton W, Misri S, Riggs W, Aghajanian J, Hertzman C. Major congenital malformations following prenatal exposure to serotonin reuptake inhibitors and benzodiazepines using population-based health data. Birth Defects Res B Dev Reprod Toxicol. 2008 Feb. 83(1):68-76. [Medline].

  6. Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of Congenital Heart Defects in Metropolitan Atlanta, 1998-2005. J Pediatr. 2008 Jul 25. [Medline].

  7. Kapoor R, Gupta S. Prevalence of congenital heart disease, Kanpur, India. Indian Pediatr. 2008 Apr. 45(4):309-11. [Medline].

  8. Axt-Fliedner R, Schwarze A, Smrcek J, et al. Isolated ventricular septal defects detected by color Doppler imaging: evolution during fetal and first year of postnatal life. Ultrasound Obstet Gynecol. 2006 Mar. 27(3):266-73. [Medline].

  9. Bahtiyar MO, Dulay AT, Weeks BP, Friedman AH, Copel JA. Prenatal course of isolated muscular ventricular septal defects diagnosed only by color Doppler sonography: single-institution experience. J Ultrasound Med. 2008 May. 27(5):715-20. [Medline].

  10. van den Bosch AE, Ten Harkel DJ, McGhie JS, et al. Feasibility and accuracy of real-time 3-dimensional echocardiographic assessment of ventricular septal defects. J Am Soc Echocardiogr. 2006 Jan. 19(1):7-13. [Medline].

  11. Alsoufi B, Karamlou T, Osaki M, et al. Surgical repair of multiple muscular ventricular septal defects: the role of re-endocardialization strategy. J Thorac Cardiovasc Surg. 2006 Nov. 132(5):1072-80. [Medline].

  12. Kadner A, Dave H, Dodge-Khatami A, et al. Inferior partial sternotomy for surgical closure of isolated ventricular septal defects in children. Heart Surg Forum. 2004. 7(5):E467-70. [Medline].

  13. Amin Z, Cao QL, Hijazi ZM. Closure of muscular ventricular septal defects: Transcatheter and hybrid techniques. Catheter Cardiovasc Interv. 2008 Jul 1. 72(1):102-11. [Medline].

  14. Aleem IS, Karamlou T, Benson LN, McCrindle BW. Transcatheter device versus surgical closure of ventricular septal defects: a clinical decision analysis. Catheter Cardiovasc Interv. 2006 Apr. 67(4):630-6. [Medline].

  15. Crossland DS, Wilkinson JL, Cochrane AD, d'Udekem Y, Brizard CP, Lane GK. Initial results of primary device closure of large muscular ventricular septal defects in early infancy using periventricular access. Catheter Cardiovasc Interv. 2008 Sep 1. 72(3):386-91. [Medline].

  16. Szkutnik M, Kusa J, Bialkowski J. Percutaneous closure of post-traumatic and congenital muscular ventricular septal defects with the Amplatzer Muscular VSD Occluder. Kardiol Pol. 2008 Jul. 66(7):715-20; discussion 721. [Medline].

  17. Vasilyev NV, Melnychenko I, Kitahori K, et al. Beating-heart patch closure of muscular ventricular septal defects under real-time three-dimensional echocardiographic guidance: a preclinical study. J Thorac Cardiovasc Surg. 2008 Mar. 135(3):603-9. [Medline].

  18. Holzer R, Balzer D, Cao QL, Lock K, Hijazi ZM. Device closure of muscular ventricular septal defects using the Amplatzer muscular ventricular septal defect occluder: immediate and mid-term results of a U.S. registry. J Am Coll Cardiol. 2004 Apr 7. 43(7):1257-63. [Medline].

  19. Kang SL, Tometzki A, Caputo M, Morgan G, Parry A, Martin R. Longer-term outcome of perventricular device closure of muscular ventricular septal defects in children. Catheter Cardiovasc Interv. 2015 May. 85 (6):998-1005. [Medline].

  20. Crossland DS, Wilkinson JL, Cochrane AD, d'Udekem Y, Brizard CP, Lane GK. Initial results of primary device closure of large muscular ventricular septal defects in early infancy using periventricular access. Catheter Cardiovasc Interv. 2008 Sep 1. 72(3):386-91. [Medline].

  21. Kim SJ, Huh J, Song JY, Yang JH, Jun TG, Kang IS. The hybrid perventricular closure of apical muscular ventricular septal defect with Amplatzer duct occluder. Korean J Pediatr. 2013 Apr. 56(4):176-81. [Medline]. [Full Text].

  22. Haponiuk I, Chojnicki M, Jaworski R, Steffek M, Juscinski J, Sroka M, et al. Hybrid approach for closure of muscular ventricular septal defects. Med Sci Monit. 2013 Jul 29. 19:618-24. [Medline]. [Full Text].

  23. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007 Oct 9. 116(15):1736-54. [Medline].

 
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