Simply select the section you would like to view from the list below: Select a section INTRODUCTION GENERAL RECOMMENDATIONS SECTION I - Atrial Septal Defect SECTION II - Ventricular Septal Defect SECTION III - Atrioventricular Septal Defect SECTION IV - Patent Ductus Arteriosus APPENDICES SECTION V - Left Ventricular Outflow Tract Obstruction... SECTION VI - Coarctation of the Aorta SECTION VII - Right Ventricular Outflow Tract Obstruction SECTION VIII - Tetralogy of Fallot SECTION IX - Ebstein Anomaly SECTION X - Marfan Syndrome SECTION XI - Complete Transposition of the Great Arteries SECTION XII - Congenitally Corrected Transposition of... SECTION XIII - Fontan Operation SECTION XIV - Single Ventricles SECTION XV - Eisenmenger Syndrome SECTION XVI - Management of Cyanotic Patients BIBLIOGRAPHY

SECTION II - VENTRICULAR SEPTAL DEFECT Part I - Background Information Only isolated VSDs will be considered. Hemodynamic Severity Grading of Isolated Ventricular Septal Defects (VSDs) in Adults Small: Pulmonary/aortic systolic pressure ratio < 0.3, and pulmonary/systemic flow ratio (Qp/Qs) < 1.4 Moderate: Systolic pressure ratio > 0.3, and Qp/Qs 1.4 to 2.2 Large: Systolic pressure ratio > 0.3, and Qp/Qs > 2.2 Eisenmenger: Systolic pressure ratio > 0.9, and Qp/Qs < 1.5 Physiological Classification of Isolated VSD in Adults Restrictive: RV pressure < LV pressure in the absence of right ventricular outflow tract obstruction. Non-restrictive: Equal right and left ventricular pressures in the absence of right ventricular outflow tract obstruction. Clinical Severity Grading of Isolated VSDs in Adults Small: Causes negligible hemodynamic changes. LV size is usually normal without any pulmonary hypertension. Moderate: Causes enlargement of left ventricle and left atrium and usually some pulmonary hypertension (reversible). Large: Results in pulmonary vascular obstructive disease and Eisenmenger physiology unless there is coexistent right Ventricular outflow tract obstruction. Pathologic/ Surgical Classification Perimembranous Bordered by fibrous continuity of an atrioventricular valve and an arterial valve, usually with inlet or outlet extension Muscular Bordered by muscle rim, usually trabecular Doubly committed Bordered by fibrous continuity of both the aortic and the pulmonary valve VSDs may co-exist with other cardiac lesions (especially valvar or subvalvar pulmonary stenosis) or result in secondary infundibular hypertrophy, right ventricular outflow obstruction and aortic regurgitation from aortic cusp prolapse. Part II - Prevalence and Genetics Doubly-committed VSDs are more common in Asian patients. Part III - History and Management of Unoperated Patients Small VSDs are associated with a relatively high risk of endocarditis but otherwise patients enjoy a normal life expectancy. Atrial arrhythmias may occur. Spontaneous closure of VSDs can still occur occasionally in adult life. Moderate VSDs are unusual in the adult but may occur when a prolapsing aortic valve cusp partially obstructs the defect. They are associated with the development of left heart dilation and shunt-related pulmonary hypertension (which often reverses with correction of the defect), and resultant congestive heart failure and atrial fibrillation, as well as the risk of endocarditis. Large VSDs without pulmonary hypertension exist in adults only when associated with obstruction to right ventricular outflow and are rare. All such patients are at risk for endocarditis. Some are cyanotic because of more severe right ventricular outflow tract obstruction at the infundibular or valvular level. VSD patients with Eisenmenger syndrome (see section XV) are at continuous risk of mortality and morbidity. Poor prognostic features are felt to be atrial flutter/fibrillation, syncope, heart failure, hemoptysis and aneurysmal dilation of proximal hypertensive pulmonary arteries which may rupture even with laminated thrombus in such dilated arteries. Five percent of VSDs develop aortic valve regurgitation. Patients with doubly-committed sub-arterial VSDs are more likely to develop aortic regurgitation from progressive prolapse of the aortic valve cusps than those with a perimembranous VSD (23). Part IV- Diagnostic Recommendations An adequate diagnostic workup: Documents the number and type(s) of VSD. Determines the size (restrictive/non-restrictive) and functional importance (left-to-right shunt estimate; left and right ventricular size/function; ventricular volume and pressure overload; pulmonary artery pressure and resistance) of the defect. Identifies other associated conditions that may influence management (aortic regurgitation; subaortic stenosis; right ventricular outflow obstruction; significant valve disease; coronary artery disease; coarctation of the aorta). The initial workup should include at a minimum: A thorough clinical assessment. ECG. Chest x-ray. Transthoracic echo-Doppler evaluation by an appropriately trained individual. The diagnostic workup may require: Oximetry. Heart catheterization (to determine pulmonary artery pressures and resistances [ reversibility using oxygen, nitric oxide and/or prostaglandins]; to assess intracardiac shunting; to evaluate associated lesions, particularly if aortic regurgitation is present; to exclude multiple VSDs). Coronary angiography in patients at risk of coronary artery disease or in patients over the age of 40 years if a surgical repair is planned. Open lung biopsy should only be considered when the reversibility of the pulmonary hypertension is uncertain from the hemodynamic data. It is potentially hazardous and should be done only at centres with substantial relevant experience in CHD. MRI occasionally to confirm the presence or absence of other associated lesions or to help define the anatomy of the aortic cusps to eliminate aortic valve prolapse. MRI can also be used to estimate Qp/Qs. Part V - Indications for Intervention The following situations warrant operative closure: The presence of a "significant" VSD [symptomatic; Qp/Qs of 2/1; pulmonary artery systolic pressure > 50 mmHg]; deteriorating ventricular function due to volume (LV) or pressure (RV) overload). Significant right ventricular outflow tract obstruction (cath gradient or mean echo gradient greater than 50 mmHg). A perimembranous or doubly committed VSD with more than mild aortic incompetence. In the presence of severe pulmonary hypertension (PAP > 2/3 SABP or pulmonary arteriolar resistance greater than 2/3 systemic arteriolar resistance), there must be a net left-to-right shunt of at least 1.5:1; or evidence of pulmonary artery reactivity when challenged with a pulmonary vasodilator (e.g. oxygen, nitric oxide and/or prostaglandins); or lung biopsy evidence that pulmonary arterial changes are potentially reversible (Heath Edwards grade II-III or less). Grade: C Level: IV Refs: 24-29 Endocarditis (especially recurrent) may be an indication for operative closure (30). Transvenous pacing should be avoided where possible in all patients with VSDs since paradoxical emboli may occur. For the same reason, venous thromboemboli from any source are a potential hazard. Grade: C Level: V Refs: 11 Part VI - Surgical/Interventional Technical Options Patients with an isolated VSD with or without associated lesions (right ventricular outflow tract obstruction, aortic valve prolapse, subaortic stenosis or infective endocarditis) should be repaired by congenital heart surgeons. Grade: C Level: V Refs: 18-19 Device closure of VSDs may be performed in the setting of isolated trabecular muscular VSDs but are still considered an experimental procedure for perimembranous VSDs (31,32). Part VII - Surgical/Interventional Outcomes Successful closure is associated with excellent survival if ventricular function is normal. Elevated pulmonary artery pressures preoperatively may progress, regress or remain unchanged post-operatively. Part VIII - Arrhythmias Atrial fibrillation may occur, especially if there has been longstanding volume overload of the left heart, or if other reasons for left atrial dilation are present. Late ventricular arrhythmias and sudden death are a potential risk especially in patients repaired late in life. (33,34). Complete heart block may also occur after surgical repair. Part IX - Pregnancy Pregnancy is well tolerated in women with small or moderate VSD and in women with repaired VSDs. Pregnancy is contraindicated in Eisenmenger syndrome because of high maternal (up to 50%) and fetal (up to 60%) mortality. Grade: C Level: V Refs: 22 Part X - Follow Up Patients with the following problems benefit from periodic evaluation by an ACHD cardiologist: Patch leaks or residual VSDs (which seldom require reoperation). Elevated pulmonary vascular resistance at the time of surgery. Aortic valve surgery. Late repair of moderate or large defects. Significant atrial or ventricular arrhythmias. Associated cardiac lesions (e.g. right ventricular outflow tract obstruction or aortic regurgitation). Endocarditis prophylaxis is recommended for 6 months following VSD closure or for life if any residual defect persists. Grade: Consensus

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