History

Fact Explanation
An asymptomatic child, referred following routine clinical examination. In small ventricular septal defects <0.5cm2, the defect is restrictive and right ventricular pressure remains normal. At birth and in the first few weeks following, the pulmonary vascular resistance remains high. As a result, the magnitude of the left to right shunt and the symptoms are minimal. [1] [2] [5] [8]
A 2-3 months child with excessive sweating (scalp sweating noticed by the mother) with feeding. In moderate to large defects. Sympathetic over-stimulation following heart failure. The symptoms appear few weeks later as the pulmonary vascular resistance decreases with time and the magnitude of the left to right shunt becomes greater. [3] [4]
Fatigue during feeding. In moderate to large defects. Feeding increases the cardiac output and sympathetic over-stimulation. It is a feature of heart failure and presents after first few weeks of life. [1] [3]
Growth faltering, loss of gained weight. In moderate to large defects. It is evident with onset of heart failure and a feature of increased calorie requirement. [1] [3] [13]
Recurrent respiratory tract infections. The left to right shunt increases the pulmonary blood flow, and thus in turn causes vascular congestion in the lungs. This acts as a predisposing factor for recurrent respiratory infections. [6] [7]
More severe forms of weight loss, shortness of breath and respiratory tract infections that presents a few weeks later than above symptoms In large defects. The symptoms are more severe but they occur a few weeks later on than the children with moderate defects. The reason behind this is in larger defects, the pulmonary vascular medial remodeling starts and happens later and slower than those with smaller defects. [8]
Chronic cough, hemoptysis, fatigue and exertional dyspnoea which appears later on. Development of pulmonary hypertension in long standing defect. Because of the high pulmonary blood flow, there is a resultant intimal proliferation of pulmonary resistance vessels. [9]
Hoarseness of cry Known as Ortners syndrome or cardiovocal hoarseness. With later stages of severe pulmonary hypertension. This is due to the compression of recurrent laryngeal nerve by the distended pulmonary artery. [10] [11] [12]
Parents noticing a bluish discoloration of the child's skin especially when crying. Onset of Eisenmengers syndrome, a more severe form of pulmonary hypertension. [14] [15]
References
  1. KLIEGMAN R, BERHAM RE, JENSON HB and STANTON BF. Nelson Textbook of Pediatrics 18th Edition. Saunders; 18 edition (25 Apr 2008)
  2. ATIK E. Small ventricular septal defect: long-term expectant clinical management. Arq Bras Cardiol. 2009 Jun;92(6):396-9, 413-6, 429-32. available at http://www.ncbi.nlm.nih.gov/pubmed/19629304
  3. CLARK BJ. Treatment of heart failure in infants and children. Heart Dis. 2000 Sep-Oct;2(5):354-61.
  4. FRAZIER A, HUNT EA and HOLMES K. Pediatric cardiac emergencies: Children are not small adults. J Emerg Trauma Shock. 2011 Jan-Mar; 4(1): 89–96
  5. TURNER SW, HUNTER S and WYLLIE JP. The natural history of ventricular septal defects. Arch Dis Child 1999;81:413-416
  6. LIU JX, WANG JH, YANG SR, LIU M, XU Y, SUN JH and YAN CY. Clinical utility of the ventricular septal defect diameter to aorta root diameter ratio to predict early childhood developmental defects or lung infections in patients with perimembranous ventricular septal defect. J Thorac Dis 2013;5(5):600-604. doi: 10.3978/j.issn.2072-1439.2013.09.05
  7. KABALKA AK. Physiologic risk factors for respiratory viral infections and immunoprophylaxis for respiratory syncytial virus in young children with congenital heart disease. Pediatric Infectious Disease Journal:January 2004 - Volume 23 - Issue 1 - pp S41-S45
  8. MASSIN MM and DESSY H.Delayed recognition of congenital heart disease. Postgrad Med J 2006;82:468–470.
  9. SIMONNAEU G et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S43-54.
  10. SENGUPTA A, DUBEY SP, CHAUDHRI D, SINHA AK and CHAKRAVARTI P. Ortner's syndrome revisited. J Laryngol Otol. 1998 Apr;112(4):377-9
  11. NAKAO M, SAWAYAMA T, SAMUKAWA M, MITAKE H, NEZUO S, FUSENO H and HASEGAWA K. Left recurrent laryngeal nerve palsy associated with primary pulmonary hypertension and patent ductus arteriosus. J Am Coll Cardiol. 1985 Mar;5(3):788-92
  12. PRADA-DELGADO O and BARGE-CABALLERO E. Ortner’s Syndrome. N Engl J Med 2011; 365:939 September 8, 2011
  13. MENON G and POSKIT EME. Why does congenital heart disease cause failure to thrive? Archives of Disease in Childhood, 1985, 60, 1134-1139
  14. IVETA S. Eisenmenger syndrome--a unique form of pulmonary arterial hypertension. Bratisl Lek Listy. 2009;110(12):757-64.
  15. SIMKOVA I et al. Clinical and hemodynamic picture of Eisenmenger syndrome. Bratisl Lek Listy. 2009;110(12):788-94.

Examination

Fact Explanation
Refer to the growth chart: poor weight gain Especially in moderate to large defects, because the calorie requirement is higher, feeding difficulties and frequent respiratory tract infections. Usually a sensitive indicator of congestive cardiac failure. [1] [4]
Profuse perspiration, evident as scalp sweating. After the onset of heart failure, sympathetic over-stimulation following increased cardiac demand. [5] [6]
Duskiness of the skin, specially when the child is crying. Before the onset of pulmonary vascular hypertension, cyanosis is usually abset but when the cardiac requirement is high, there can be a mild duskiness of the skin colour depending on the size of the defect, more obvious in larger ones.[1]
Mild tachypnoea In moderate to large defects. Due to heart failure and pulmonary congestion. [1] [5] [6]
Tachycardia In moderate to large defects. Due to heart failure and sympathetic over-stimulation. [1] [5] [6]
Systolic fluid thrill but apex is not displaced. In small defects. The turbulance that is caused by shooting of blood through the septal defect can be felt as a thrill at the apex. But the shunt is restrictive so the cardiac remodeling is usually minimal. [1] [7]
Hyperdynamic precordium: parasternal lift and apical thrust with a systolic thrill. In large defects. The volume overload causes ventricular dilatation and remodeling. [1] The cardiomegaly is also a feature of heart failure. [5] [6]
A localized harsh, holosystolic murmur which is best heard at the lower left sternal border, detected after 4-8 weeks of age or later. The murmur might end before the 2nd sound. In small defects which are <0.5 cm2. Usually other vital signs are normal. Throughout the systole, when the pressures of the both ventricles rise with myocardial contraction, the blood gushes from the left ventricle to the right through the narrow defect, giving rise to a localized, squeaky systolic murmur. The murmur becomes apparent only after the fall of pulmonary vascular resistance, which takes a few weeks after birth. [1] [2] [3]
A low pitch holosystolic murmur which is best heard at the lower left sternal border. In larger defects, there the pressure difference between the right and left ventricles is small and thus the smaller turbulence. [1]
A mid diastolic rumble at the mitral area. Due to the functional mitral stenosis. [1]
A loud pulmonic component of the second heart sound, and a systolic ejection murmur may be heard over the left sternal border. Fixed or paradoxical splitting of the second heart sound may be there. Due to secondary pulmonary hypertension. [8] [9] [10]
Cyanosis, bleeding manifestations and symptoms of deep vein thrombosis, heart failure along with pulmonary hypertension. Development of Eisenmengers syndrome. [11] [12]
Disappearing murmur. In small VSDs, majority of the defects close between the age of 1-5 years. [3] [7] [13]
References
  1. KLIEGMAN R, BERHAM RE, JENSON HB and STANTON BF. Nelson Textbook of Pediatrics 18th Edition. Saunders; 18 edition (25 Apr 2008)
  2. ATIK E. Small ventricular septal defect: long-term expectant clinical management. Arq Bras Cardiol. 2009 Jun;92(6):396-9, 413-6, 429-32. available at http://www.ncbi.nlm.nih.gov/pubmed/19629304
  3. TURNER SW, HUNTER S and WYLLIE JP. The natural history of ventricular septal defects. Arch Dis Child 1999;81:413-416
  4. MENON G and POSKIT EME. Why does congenital heart disease cause failure to thrive? Archives of Disease in Childhood, 1985, 60, 1134-1139
  5. FRAZIER A, HUNT EA and HOLMES K. Pediatric cardiac emergencies: Children are not small adults. J Emerg Trauma Shock. 2011 Jan-Mar; 4(1): 89–96
  6. CLARK BJ. Treatment of heart failure in infants and children. Heart Dis. 2000 Sep-Oct;2(5):354-61.
  7. ATIK E. Small ventricular septal defect: long-term expectant clinical management. Arq Bras Cardiol. 2009 Jun;92(6):396-9, 413-6, 429-32. available at http://www.ncbi.nlm.nih.gov/pubmed/19629304
  8. SIMONNEAU G et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):5S-12S.
  9. GALIE N et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2009 Oct;30(20):2493-537.
  10. VAN ALBADA ME and BERGER RM. Pulmonary arterial hypertension in congenital cardiac disease--the need for refinement of the Evian-Venice classification. Cardiol Young. 2008 Feb;18(1):10-7.
  11. SIMKOVA I et al. Clinical and hemodynamic picture of Eisenmenger syndrome. Bratisl Lek Listy. 2009;110(12):788-94.
  12. IVETA S. Eisenmenger syndrome--a unique form of pulmonary arterial hypertension. Bratisl Lek Listy. 2009;110(12):757-64.
  13. RUANGRITNAMCHAI C, KHOWSATHIT P and PONGPANICH B. Spontaneous closure of small ventricular septal defect first six months of life.J Med Assoc Thai. 1993 Oct;76 Suppl 2:63-71.

Differential Diagnoses

Fact Explanation
Atrioventricular septal defect Unlike ventricular septal defect where the long term prognosis is good, especially in small defects [10], J, atrioventricular canal defect is a complex cardiac defect, often associated with other syndromic diseases, with a higher mortality. [11] [12] Some patients are asymptomatic independent of the sixe of the defect and some rapidly go into heart failure. The presentation can be similar to the ventricular septal defect, as there is a left to right shunt and pulmonary vascular congestion. The murmur heard in examination can be similar to the atrio-septal defect. [13] [14]
Patent ductus arteriosus The ductus arteriosus is essentially open in the fetal life and closes at birth. Patent ductus arteriosus occurs when a persistent communication occurs between the descending thoracic aorta and the pulmonary artery due to failure of normal physiologic closure of the fetal ductus. The patients, like in VSD, can be missed at birth and be asymptomatic for sometime before presentation. There can be symptoms of exertion intolerance after first few weeks of life. Since a large volume of blood enters the pulmonary circulation, there can be features of pulmonary vascular congestion, e.g. recurrent pneumonia. Features of heart failure are also can be presentations, such as tachypnea, inability or difficulty with feeding, diaphoresis, and weight loss or no weight gain. The murmur in the neonate can be rather a systolic one than the characteristic continuous one. [5] [6]
Infundibular pulmonary stensosis Rather than an obstruction at the valvular level, this refers to an outflow tract obstruction from the right ventricle within the body of it. Most patients are asymptomatic despite of the severity of the stenosis, and diagnosed with an ejection systolic murmur and a soft pulmonic component of the second heart sound at routine examinations. Some patients with severe obstructions, can present with cyanosis and frank heart failure soon after birth. [15] [16] [17]
Gerbode defect Gerbode defect is a rarer form of defect and a collective name for a cardiac defect that has two components, a deficiency of the atrio-ventricular membranous septum,and shunting initially through a ventricular septal defect, with atrial shunting occurring through a deficiency in the septal leaflet of the tricuspid valve. [1] It has been even more rarely reported folowing bacterial endocarditis and myocardial infarction. [2] [3] The easiest and the best diagnostic tool is echocardiography. [4]
References
  1. KELLE AM, YOUNG L, KAUSHAL S, DUFFY CE, ANDERSON RH and BACKER CL. The Gerbode defect: the significance of a left ventricular to right atrial shunt. Cardiol Young. 2009 Nov;19 Suppl 2:96-9.
  2. VELEBIT V et al. “Acquired” Left Ventricular-to-Right Atrial Shunt (Gerbode Defect) after Bacterial Endocarditis. Tex Heart Inst J 1995;22:100-2.
  3. CORTEZ-DIAS N et al. Acquired left ventricular-to-right atrial shunt (Gerbode defect) due to infective endocarditis. Rev Port Cardiol. 2009 Jun;28(6):735-9.
  4. PATANE S, MARTE F and DI BELLA G. Echocardiographic diagnosis of syndrome of left ventricular-right atrial shunt (Gerbode defect). Int J Cardiol. 2008 Aug 29;128(3):e85-6.
  5. DICE JE and BHATHIYA J. Patent Ductus Arteriosus: An Overview. J Pediatr Pharmacol Ther. 2007 Jul-Sep; 12(3): 138–146.
  6. SCHNEIDER DJ. The patent ductus arteriosus in term infants, children, and adults. Semin Perinatol. 2012 Apr;36(2):146-53.
  7. JOHNSON AM. Hypertrophic infundibular stenosis complicating simple pulmonary valve stenosis. Br Heart J. Jul 1959; 21(3): 429–439.
  8. WEYMAN AE, DILLON JC, FEIGENBAUM H and CHANG S. Echocardiographic differentiation of infundibular from valvular pulmonary stenosis. Am J Cardiol. 1975 Jul;36(1):21-6.
  9. NEUFELD HN, ONGLEY PA, and EDWARDS JE. Combined congenital subaortic stenosis and Infundibular pulmonary stenosis. Br Heart J. Nov 1960; 22(5): 686–690.
  10. GABRIEL HM et al. Long-term outcome of patients with ventricular septal defect considered not to require surgical closure during childhood. J Am Coll Cardiol. 2002;39(6):1066-1071.
  11. MACRIS MP, OTT DA and COOLEY DA. Complete Atrioventricular Canal Defect. Tex Heart Inst Jv.19(3); 1992
  12. DIGILIO MC, MARINO B, TOSCANO A, GIANNOTTI A, DALLAPICCOLA B Atrioventricular canal defect without Down syndrome: a heterogeneous malformation. Am J Med Genet. 1999 July 16; 85(2): 140–146.
  13. KLIEGMAN R, BERHAM RE, JENSON HB and STANTON BF. Nelson Textbook of Pediatrics 18th Edition. Saunders; 18 edition (25 Apr 2008)
  14. MINICH LL et al. Partial and Transitional Atrioventricular Septal Defect Outcomes. Ann Thorac Surg. 2010 February; 89(2): 530–536.
  15. LITTLE JB, LAVENDER JP and DESANCTIS RW. The Narrow Infundibulum in Pulmonary Valvular Stenosis :Its Preoperative Diagnosis by Angiocardiography. Circulation. 1963;28:182-189
  16. JOHNSON AM, Hypertrophic infundibular stenosis complicating Simple pulmonary valve stenosis. Br Heart J. Jul 1959; 21(3): 429–439.
  17. MARTON T et al. Pulmonary stenosis and reactive right ventricular hypertrophy in the recipient fetus as a consequence of twin-to-twin transfusion. Prenat Diagn. Jun 2001;21(6):452-6.

Investigations - for Diagnosis

Fact Explanation
Chest radiography: erect, in full inspiration, postero-anterior and views. In small defects, the chest Xray film will reveal a normal sized heart and normal pulmonary vascular markings. In larger defects, it will show cardiomegaly. prominent main pulmonary artery segment with increased pulmonary vascular markings and enlarged left atrium. Once pulmonary hypertension develops, there will be right ventricular hypertrophy and reduced pulmonary vascularity in the outer third of the lung fields. [1] [2] [3]
Echocardiograpghy Echocardiogram can be used to assess the defct and also the percutaneous treatment methods. [4]. In context of the defect, Echocardiogram can be used to assess the number of drfects and their positions, and also the associated other cardiac defects. [5] The rare swiss cheese septum, the multiple defects in the ventricular septum also can be identified. [6] In addition, the complications, presence of heart failure and the ejection fraction also can be assessed. 3dimensional echo is better than 2 dimensional. [7] [8]
Magnetic Resonance Imaging MRI can be useful but used in infrequent requirements where most of the time reason is the Echocardiography is not adequate. But the systemic and pulmonary blood flow has been well described from MRI so, to assess the severity of left-to-right shunt, MRI is more useful. [9] [10] [11]
Doppler color flow ultrasonography and two-dimensional mapping. It is a valuable aid in the diagnosis of VSD and may be one reason for the observed increase in the incidence of VSD [14]. It also allows the physician to assess overall prognosis by deciding the placement of the defect and presence of heart failure. It also can be used to follow up with time. [14]
Electrocardiography The ECG changes depend on the cardiac manifestations by the defect, and that in turn essentially by the size of the defect. Small defects show no ECG changes. Moderate VSDs with significant left-to-right shunts with volume overload, left ventricular hypertrophy or even biventricular hypertrophy is the expected feature in ECG. The latter is known as Katz-Wachtel phenomenon. [15] Left axis deviation is also a common finding. In patients with large VSDs and equal ventricular pressures, right ventricular hypertrophy is expected. In patients with large pulmonary blood flow, left atrial hypertrophy is evidenced. [12] [13]
Cardiac catheterization and angiography. In the previous studies it has been found that the sensitivity and specificity of this is not significant from that of Echocardiography. [16]
References
  1. SIMON G, SYMONS JC and JOSEPH M. The plain chest radiograph in infants with an isolated ventricular septal defect. Clin Radiol. 1977 May;28(3):323-6.
  2. Somerville J and Grech V The chest x-ray in congenital heart disease 2. Images Paediatr Cardiol. 2010 Jan-Mar; 12(1): 1–8.
  3. KOCZYŃSKI A. [Value of chest x-ray films in the diagnosis of congenital heart defects in infants]. Probl Med Wieku Rozwoj. 1982;11:121-31.
  4. PENNY DJ, VICK GW 3rd. Ventricular septal defect. Lancet 2011; 377:1103.
  5. KARDON RE, CAO QL, MASANI N, et al. New insights and observations in three-dimensional echocardiographic visualization of ventricular septal defects: experimental and clinical studies. Circulation 1998; 98:1307.
  6. SABOO AR, VIJAYKUMAR R, MALIK S and WARKE C. Swiss cheese ventricular septal defect with myocarditis - a rare coexistence in a neonate. J Postgrad Med. 2012 Apr-Jun;58(2):147-9
  7. VAN DEN BOSCH AE et al. Feasibility and accuracy of real-time 3-dimensional echocardiographic assessment of ventricular septal defects. J Am Soc Echocardiogr 2006; 19:7
  8. CHENG TO, XIE MX, WANG XF, et al. Real-time 3-dimensional echocardiography in assessing atrial and ventricular septal defects: an echocardiographic-surgical correlative study. Am Heart J 2004; 148:1091
  9. LOWELL DG, TURNER DA, SMITH SM, BUCHELERES GH, SANTUCCI BA, GRESICK RJ JR and MONSON DO. The detection of atrial and ventricular septal defects with electrocardiographically synchronized magnetic resonance imaging. Circulation.1986; 73: 89-94.
  10. BREMERICH J, REDDY GP and HIGGINS CB. MRI of supracristal ventricular septal defects. J Comput Assist Tomogr. 1999 Jan-Feb;23(1):13-5.
  11. YOSHIMURA N, HORI Y, HORII Y, SUZUKI H, HASEGAWA S, TAKAHASHI M and WATANABE H. Comparison of magnetic resonance imaging with transthoracic echocardiography in the diagnosis of ventricular septal defect-associated coronary cusp prolapse. JACC Cardiovasc Interv. 2011 Dec;4(12):1326-34
  12. GUO M, HUANG MN, BAI Z and HSIEH KS. Important ECG diagnosis-aiding indices of ventricular septal defect children with or without congestive heart failure. Stat Med. 2001 Apr 15;20(7):1125-41.
  13. VINCE DJ and KEITH JD. The Electrocardiogram in Ventricular Septal Defect. Circulation.1961; 23: 225-240.
  14. MEHTA AV, GOENKA S, CHIDAMBARAM B, HAMATI F. Natural history of isolated ventricular septal defect in the first five years of life. Tenn Med. 2000 Apr;93(4):136-8.
  15. KHAIRY P and MARELLI AJ. Clinical Use of Electrocardiography in Adults With Congenital Heart Disease. Circulation. 2007; 116: 2734-2746
  16. MAGEE AG, BOUTIN C, MCCRINDLE BW and SMALLHORN JF. Echocardiography and cardiac catheterization in the preoperative assessment of ventricular septal defect in infancy. Am Heart J. 1998 May;135(5 Pt 1):907-13.

Investigations - Followup

Fact Explanation
Echocardiography To follow up the course of the defect and complications. [1] [2] [3] [4] It also can be used in follow up after corrective surgery. [5]
References
  1. KARDON RE, CAO QL, MASANI N, et al. New insights and observations in three-dimensional echocardiographic visualization of ventricular septal defects: experimental and clinical studies. Circulation 1998; 98:1307.
  2. TOMITA H, ARAKAKI Y, YAGIHARA T and ECHIGO S. Incidence of spontaneous closure of outlet ventricular septal defect. Jpn Circ J. 2001 May;65(5):364-6.
  3. CHANTEPIE A, LUKSENBERG S, VAILLANT MC, POTTIER JM, MAGONTIER N, DESPERT F and NEVILLE P. [Evolution of ventricular septal defects. Relation to echocardiographic anatomy]. Arch Mal Coeur Vaiss. 1999 May;92(5):623-8.
  4. VAILLANT MC, CHANTEPIE A, CHELIAKINE C, NASHASHIBI M, POTTIER JM and LAUGIER J. [Contribution of two-dimensional echography in predicting spontaneous closure of interventricular defects in infants]. Arch Mal Coeur Vaiss. 1992 May;85(5):597-601.
  5. KLITSIE LM, ROEST AA, BLOM NA, and TEN HARKEL AD. Ventricular performance after surgery for a congenital heart defect as assessed using advanced echocardiography: from doppler flow to 3D echocardiography and speckle-tracking strain imaging. Pediatr Cardiol. 2014 Jan;35(1):3-15.

Management - General Measures

Fact Explanation
Small VSD- No surgical management required. Observation with maintaining adequate oral hygiene is the management of choice. Studies have proved that a favorable evolution of the small VSD at long-term follow-up does not require surgical intervention, with concerns regarding the strict antibiotic prophylaxis. [1] The overall clinical outcome of muscular VSD is better than that of the perimembranous type. [2] Surgical closure does not appear to be required during childhood as long as left-to-right shunt is <50% and signs of LV volume overload are absent, when pulmonary arterial pressure is not elevated, and no VSD-related Aortic Regurgitation or symptoms are present. [3]
Antibiotic prophylaxis with oral penicillin. Prophylaxis is less necessary for patients who have documented complete closure of VSD. [4] Fully repaired VSDs do not require long term antibiotic prophylaxis. [5] The incidence of bacterial endocarditis with severity of the VSD is not clearly established, so unless the patients are going for dental or surgical procedures, it's not advisable to give long term antibiotic prophylaxis. [5]
High calorie diet plan. Especially for the patients with heart failure or recurrent infections, and resultant growth faltering. Diet supplemented with high calorie food items will ensure adequate growth.
References
  1. ATIK E. Small ventricular septal defect: long-term expectant clinical management. Arq Bras Cardiol. 2009 Jun;92(6):396-9, 413-6, 429-32.
  2. MEHTA AV, GOENKA S, CHIDAMBARAM B and HAMATI F. Natural history of isolated ventricular septal defect in the first five years of life. Tenn Med. 2000 Apr;93(4):136-8.
  3. GABRIEL HM, HEGER M, INNERHOFER P, ZEHETGRUBER M, MUNDIGLER G, WIMMER M, MAURER G and BAUMGARTNER H. Long-term outcome of patients with ventricular septal defect considered not to require surgical closure during childhood. J Am Coll Cardiol. 2002 Mar 20;39(6):1066-71.
  4. GERSONY WM and HAYES CJ. Bacterial endocarditis in patients with pulmonary stenosis, aortic stenosis, or ventricular septal defect.Circulation [1977, 56(1 Suppl):I84-7]
  5. National Institute for Health and Clinical Excellence Guidelines (64). Prophylaxis against infective endocarditis: Antimicrobial prophylaxis against infective endocarditis in adults and children undergoing interventional procedures. March 2008

Management - Specific Treatments

Fact Explanation
Diuretics e.g. furosemide to relieve pulmonary congestion, reduce afterload and preload in cardiac failure. It is used as mainstay of treatment. [3] [4] [5]
Angiotensin-converting enzyme (ACE) inhibitors e.g. captopril and enalapril ACE inhibitors reduce both the systemic and pulmonary pressures, thereby reducing the left-to-right shunt. [6] [7]
Cardiac glycosides-Digoxin This may be indicated if symptoms do not respond to diuresis or ACE inhibition. [4] [3]
Direct surgical repair Using cardiopulmonary bypass is the preferred surgical therapy. The long term outcome is better if the surgical correction is performed early. [8] Caution in infants below 6months of age. [9]
Transatrial surgical approach. In perimembranous and inlet VSDs [10]
Transcatheter therapy An experimental method where the VSD of perimembranous is repaired surgically and the VSDs in muscular septum are closed with a transcatheter device. This may be used for multiple VSDs [11]
Surgical correction of regurgitant aortic valves if present. Patients with perimembranous or muscular VSDs propose a higher risk for aortic valve prolapse and regurgitation, and also once a milder form of regurgitation has started it progresses rapidly to a severe form. [1] Patients with perimembranous VSDs with AVP should be followed with serial echocardiography. [2]
References
  1. EROĞLU AG, OZTUNÇ F, SALTIK L, DEDEOĞLU S, BAKARI S and AHUNBAY G. Aortic valve prolapse and aortic regurgitation in patients with ventricular septal defect. Pediatr Cardiol. 2003 Jan-Feb;24(1):36-9.
  2. TWEDDELL JS, PELECH AN, and FROMMELT PC. Ventricular septal defect and aortic valve regurgitation: pathophysiology and indications for surgery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006:147-52.
  3. LOWRIE L. Diuretic therapy of heart failure in infants and children. Prog Pediatr Cardiol. 2000 Nov 4;12(1):45-55.
  4. ROSS RD. Medical management of chronic heart failure in children. Am J Cardiovasc Drugs. 2001;1(1):37-44.
  5. CLARK BJ 3rd. Treatment of heart failure in infants and children. Heart Dis. 2000 Sep-Oct;2(5):354-61.
  6. MOMMA K. These medications reduce both the systemic and pulmonary pressures (the latter to a greater degree), thereby reducing the left-to-right shunt. Paediatr Drugs. 2006;8(1):55-69.
  7. Lee GJ, Cohenand R and Cleary JP. Angiotensin Converting Enzyme Inhibitor (ACEI)-Induced Acute Renal Failure in Premature Newborns with Congenital Heart Disease. J Pediatr Pharmacol Ther 2010 Oct-Dec: 15 (4)
  8. ROOS-HESSELINK JW, MEIJBOOM FJ, SPITAELS SE, VAN DOMBURG R, VAN RIJEN EH, UTENS EM, BOGERS AJ and SIMOONS ML. Outcome of patients after surgical closure of ventricular septal defect at young age: longitudinal follow-up of 22-34 years.Eur Heart J. 2004 Jun;25(12):1057-62.
  9. ANDERSON BR, STEVENS KN, NICOLSON SC, GRUBER SB, SPRAY TL, WERNOVSKY G andGRUBER PJ. Contemporary outcomes of surgical ventricular septal defect closure. J Thorac Cardiovasc Surg. 2013 Mar;145(3):641-7.
  10. MERIN G, ARYANPUR I, SIMCHA A and SHAPIRA T. Transatrial closure of ventricular septal defect in corrected transposition of the Great Arteries. Chest. 1976 Feb;69(2):241-3.
  11. THANOPOULOS BD, TSAOUSIS GS and PAPHITIS C. Transcatheter closure of perimembranous ventricular septal defects with the Amplatzer asymmetric ventricular septal defect occluder: preliminary experience in children. Heart. Aug 2003; 89(8): 918-922.