Atrioventricular Canal Defect

Dr. Md. Rezwanul Hoque

MBBS, MS, FCPS, FRCSG, FRCSEd

Associate Professor Department of Cardiac surgery BSMMU, Dhaka, Bangladesh

Atrioventricular Septal Defect

Definition A deficiency or absence of septal tissue immediately above & below the normal level of the AV valves including the region normally occupied by the AV septum in heart with two ventricle and the AV valves are abnormal to a varying degree. Aortic valve is elevated, deviated anteriorly due to absence of usual wedged position of aortic valve above the AV valve. It is caused by an abnormal or inadequate fusion of the superior and inferior endocardial cushion with the mid portion of the atrial septum and the muscular portion of the ventricular septum. Synonyms: AV septal defect, AV canal defect, AV defect, ECD (Endocardial cushion defects), Ostium primum atrial septal defects, Common AV orifice

Atrioventricular Septal Defect

Historical note Rogers, Edwards : Recognized morphology of ostium primum ASD in 1948 Wakai, Edwards : Term of partial and complete AV canal defect in 1956 Bharati & Lev : Term of intermediate & transitional in 1980 Ugarte : Term of leaflet bridging ventricular septum in 1976 Rastelli : Described the morphology of common anterior leaflet in 1966 Lillehei : 1st repair of complete AV canal defect using cross circulation in 1954 Kirklin, Watkins, Gross : Open repair using oxygenator

Sellke: Sabiston & Spencer Surgery of the Chest, 7th ed., Copyright 2005 Saunders, An Imprint of Elsevier

Types of AV septal defect

Spectrum of disease, arbitrarily divided into- Partial, Transitional & Complete AV canal defect.
Partial AV canal defects -Large ostium primum ASD, cleft mitral valve between the left superior and inferior leaflets, no interventricular communication, two distinct AV valve orifices. Transitional AV canal defect -An intermediate form of AVSD having two distinct left AV valve and right AV valve orifices but also has both an ASD just above and a ventricular septal defect (VSD) just below the AV valves. The VSD in this intermediate form of AVSD is often restrictive. Although these AV valves in the intermediate form do form two separate orifices, they remain abnormal valves.. Complete AV canal defects -have both defects in the atrial septum just above the AV valves and defects in the ventricular septum (non- restrictive inlet VSD)just below the AV valves. In complete AVSD, the AV valve is one valve that bridges both the right and left sides of the heart, creating superior and inferior bridging leaflets.
Sellke: Sabiston & Spencer Surgery of the Chest, 7th ed., Copyright 2005 Saunders, An Imprint of Elsevier

Morphology

AV valve apparatus- 5-6 leaflets Left superior(LSL), left inferior(LIL), left lateral(LLL), the former two represents a nterior mitral leaflet. Right superior(RSL), right inferior(RIL), right lateral(RLL) leaflet. In partial AVSD, leaflets fuse on either side of ventricular crest, VSD may or may not be present In complete AVSD, LSL+RSL forms anterior bridging leaflet, LIL+RIL fuse to form posterior bridging leaflet, VSD always present. The degree of bridging and attachment of this bridging leaflet to underlying misplaced papillary muscle determines Rastellis classification(1966). The AV node and the conduction bundle is displaced inferiorly.(Lev)

Complete AVSD
In complete atrioventricular septal defect, a single atrioventricular valve annulus, a common atrioventricular valve, and a defect of the inlet ventricular septum are observed. The common AV valve consists of at least 4 leaflets. These include the anterior and posterior bridging leaflets and 2 lateral leaflets. The anterior leaflet may be further subdivided to produce a total of 5 leaflets. The classification system initially described by Rastelli et al is used to describe the morphology of the atrioventricular valve.

Rastelli type A valve (a)

The anterior leaflet is divided into 2 portions of approximately equal size. The lateral portions of this leaflet attach to the anterior papillary muscles in each ventricle. Chordae tendineae attach the medial portion of this leaflet to the crest of the ventricular septum or slightly to the right ventricular side. Interventricular communication may occur between the anterior and posterior bridging leaflets and underneath the anterior leaflet in the interchordal spaces.

Rastelli type B valves The rarest type, the anterior bridging leaflet is divided but overhangs the ventricular septum more so than in type A valves.

The chordae from the medial portion of the divided anterior leaflet have no direct insertion to the ventricular septum but rather insert onto an anomalous papillary muscle positioned in the right ventricle near the ventricular septum.
Because of the lack of chordal insertions to the septum, free interventricular communication occurs beneath the anterior leaflet.

Rastelli type C valve The anterior bridging leaflet is larger and overhangs the septum more so than with a type A and type B valves. It is not attached in its mid portion to the ventricular septum or elsewhere and is referred to as being free floating. Free interventricular communication also occurs underneath this valve leaflet.

The superior cushionderived leaflet bridges the ventricular septum and attaches to the Papillary muscle of the conus at Its rightmost extent. A right superior leaflet (RSL) Typically attaches to the papillary muscle of the conus and to the anterior papillary muscle of the right ventricle (RV), and a right lateral leaflet (RLL) attaches to the anterior papillary muscle of the RV and to the posterior papillary muscle of the RV. The inferior cushionderived bridging leaflet is usually cleft, giving the appearance of a right inferior Leaflet ( RIL) and a left inferior leaflet (LIL)

Position of conduction tissue

The location of the atrioventricular (AV) node and the conduction tissue. A, Normal heart. Note the location of the AV node at the tip of the triangle of Koch. B, AV canal heart: The AV node is now located within the nodal triangle, not at the tip of the triangle of Koch. The coronary sinus, AV node, and bundle of His are displaced inferiorly compared with the normal heart. RA, Right atrium; RBB, right bundle branch; RV, right ventricle.
Kertesz NJ: The conduction system and arrhythmias in common atrioventricular canal. Prog Pediatr Cardiol 10: 153159, 1999.)

Anatomical variability:
Shortened dimension of the inlet
septum-to-ventricular apex, giving the interventricular septum a scooped-out appearance. This deficiency in the inlet septum is typically deeper in complete AV canal defects than in partial AV canal defects. Lengthened dimension of the outlet septum-to-ventricular apex, resulting in a goose-neck appearance and anterior displacement of the left ventricular (LV) outflow tract. Although the LV outflow tract is narrowed, true LV outflow tract obstruction (LVOTO) is rare. In the normal heart, the inlet septum-toventricular apex length and the outlet septum-to-ventricular apex length are equal.

1. Normal 2. AVSD 3. After repair Absence of the usual wedged position of the aortic valve between the AV valves, caused by maldevelopment of the endocardial cushions. This results in elevation and anterior deviation of the aortic valve. Apical displacement of the attachments of the AV valves to the crest of the interventricular septum, caused by the deficiency in the inlet septum.

Atrioventricular septum
That portion of cardiac septum which lies between the right atrium and the left ventricle. It consists of a superior membranous portion and an inferior muscular portion. The atrioventricular septum is apparent because the septal attachment of tricuspid valve is more apical than the mitral valve. The AV node lies in the atrial septum adjacent to the junction between the membranous and muscular portions of atrioventricular septum, and His bundle passes toward the right trigone between these two components

Embryology
Faulty development of the endocardial cushions, which represent the primordia of the atrioventricular Septum and atrioventricular valves, plays a central role in the development of atrioventricular septal defects.
Complete failure of fusion of the endocardial cushions results in deficiency of the inlet portion of the interventricular septum, a common atrioventricular valve annulus and common AV valve, as well as deficiency of the inferior (primum) portion of the atrial septum.

Morphology of A-V Septal Defect (I)

1. Interatrial communication 1) Ostium primum ASD 2) Common atrium
entire limbus & fossa ovalis are absent

3) Unusual AV valve combination

4) Accessory orifice ; 5 %
5) Single papillary m ; 5 % 4. Ventricle; hypoplasia in 7% 5. Septal malalignment

3) Absence of interatrial shunt

rarely, due to complete attachment of AV valve tissue to atrial septum

2. Interventricular communication 1) Partial form 2) Complete form 3. AV valves 1) Two AV valve orifice 2) Common AV valve orifice

6. LVOT or inflow obst.

7. Conduction system; LAD

Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

Morphology of AV Septal Defect (II)

1. Major associated cardiac anomalies 1) PDA(10%) 2) TOF(10%) 3) DORV(3%) 4) TGA(rarely) 5) Unroofed coronary sinus with Lt. SVC (6%, frequent in common atrium) 2. Minor associated cardiac anomalies 1) ASD 2) Unroofed coronary sinus without Lt. SVC 3) Partially unroofed coronary sinus 4) Azygos extension of IVC 3. Pulmonary vascular disease ; earlier onset than VSD 4. Down syndrome 1) Rare in partial form & common in complete form (75%) 2) Lt-sided obstruction & associated anomalies less common 3) Frequent advanced pulmonary disease
Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

Atrioventricular Septal Defect

LV Outflow & Inflow Obstruction
Incidence
1% in unoperated cases Higher incidence after operation

Etiology
1. Elongation & narrowing due to more extensive area of direct fibrous continuity aortic valve & LSL 2. Short, thick chordae that anchor to the crest of ventricular septum 3. Bulging of anterolateral muscle bundle(m. of Moulart) 4. Morphologically discrete subaortic membrane or excrescences of aortic valve orifice 5. Abnormally positioned papillary muscle
Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

Causes
Trisomy 21 (Down syndrome) is the most frequent, others - Trisomy 13 and Trisomy 18, Interstitial deletion on chromosome 16. * In children with Down syndrome, AV canal defects are seen in 2025% or a 1000fold increased risk when compared with the incidence in the general population.
Sellke: Sabiston & Spencer Surgery of the Chest, 7th ed., Copyright 2005 Saunders, An Imprint of Elsevier

May be a component part ofDandy-Walker malformation, Heterotaxy syndromes (asplenia and occasionally with polysplenia). Associated conditionTAPVC Ebstein anomaly. DiGeorge syndrome

Frequency

Atrioventricular septal defects account for 2-9% of congenital heart diseas e in various series. Most investigators report a prevalence rate in the rang e of 3-5%.

The male-to-female distribution of atrioventricular septal defect is approximately equal.

The incidence of atrioventricular septal defect is higher among stillborn infants, likely due to the higher number of chromosomal and other genetic anomalies in this group. The pooled frequency of atrioventricular septal defects from several series of congenital heart disease in stillborn infants was about 7%.

Atrioventricular Septal Defect

Pathophysiology
Partial AV canal defects result from the failure of the endocardial cushions to meet the septum primum producing a low-lying (ostium primum) defect in the atrial septum ; a cleft mitral valve is also usually present.

Complete AV canal defects result from maldevelopment of the endocardial cushions, producing a single, common AV valve & VSD in addition to an ostium primum ASD. The degree of left-to-right shunting through the atrial defect is determined by the size of the communication and the relative compliance of the 2 atria and ventricles. Ventricular compliance is affected by the level of pulmonary vascular resistance (PVR). Pathophysiology results from left-to-right shunting at the atrial and/or ventricular level as well as AV valve insufficiency, producing pulmonary overcirculation and congestive heart failure, particularly during early infancy.
http://emedicine.medscape.com/article/894813-overview

Pathophysiology( cont.)
Patients with little atrioventricular valve regurgitation and high pulmonary vascular resistance (PVR) are asymptomatic early in life, and their condition may be difficult to diagnose. These patients occasionally remain relatively asymptomatic until their second or third decade, when they develop increasing cyanosis from advanced pulmonary vascular disease. In most cases, the PVR decreases normally over the first 6 weeks of life, and the patient develops a large left-to-right shunt through both the atrial and ventricular defects, resulting in congestive heart failure (CHF). Patients with clinically significant atrioventricular valve regurgitation may also have signs of CHF, such as tachypnea, excessive sweating, and failure to appropriately gain weight.

Mortality/Morbidity

Patients with complete atrioventricular septal defect typically develop tachypnea, respiratory tract infection and failure to thrive in the first few months of life. Patients may survive past the first few years of life without surgical intervention if the PVR remains elevated, although they may develop irreversible pulmonary vascular obstructive disease (PVOD) at a rapid rate.

In patients with a nonrestrictive VSD component, pulmonary vascular disease (Eisenmenger syndrome) eventually occurs unless the VSD component is surgically closed. Cyanosis occurs when patients develop some degree of right-to-left shunt at either atrial or ventricular levels. Although patients' quality of life may be impaired at this point, their life expectancy may be 20-50 years.

Clinical History
Tachypnea, repeated respiratory infections, poor feeding, and failure to thrive are frequent symptoms in patients with complete atrioventricular septal defect (AVSD) and large left-to-right shunts. These symptoms are usually present by 6-8 weeks and due to blood flow through the large interventricular communication with or without incompetence of the common atrioventricular valve. Pulmonary vascular disease results from damage caused by excessive pulmonary flow and elevated pulmonary artery pressure due to the large ventricular septal defect (VSD). Irreversible pulmonary vascular disease may be present by age 2 years or, in rare cases, earlier. Physical General physical examination may show characteristics of Down syndrome, including flat facial profile, upslanting palpebral fissures, prominent inner epicanthal folds, Brushfield spots, protuberant tongue, abnormal palmar creases, and fifth finger clinobrachydactyly. Inspection may reveal pallor or Harrison grooves (horizontal depression along lower border of chest at diaphragm insertion site due to chronic tachypnea). Failure to thrive is common due to excessive metabolic cardiovascular requirements and poor caloric intake (due to tachypnea) is common.

Cardiac examination
The cardiac examination is remarkable for and overactive precordium. The volume and pressure overload on the right ventricle result in a prominent systolic heave along the left sternal border and subxiphoid regions. The pulmonary component of the second heart sound may be palpable at the left second intercostal space. Regurgitation of the atrioventricular valve may uncommonly result in a palpable apical thrill. The first heart sound is single and often accentuated. The second heart sound is narrowly split, with an accentuated pulmonary component. A crescendo-decrescendo murmur may be audible at the upper left sternal border due to increased blood flow through a normal pulmonary valve. A mid diastolic rumble may be audible at the lower left sternal border and apex due to the Increased flow across the common atrioventricular valve. A holosystolic murmur is often appreciated at the apex due to atrioventricular valve insufficiency. Because the VSD in complete atrioventricular septal defect is large and unrestrictive, it is not associated with a murmur.

Cardiac examination- cont.

When pulmonary vascular resistance (PVR) is elevated, the systolic murmur may not be prominent, and the diastolic rumble may disappear, reflecting less left-to-right shunt.
This finding can occur in the infant in whom PVR has never fallen or in the older child with developing pulmonary vascular obstructive disease (PVOD), for whom the improvement in congestive heart failure (CHF) symptoms is an ominous finding. In patients with advanced PVOD, the left parasternal impulse is prominent, S2 may be palpable, and the systolic murmur may be soft and short.

A high-pitched decrescendo diastolic murmur of pulmonary insufficiency (Graham Steel murmur) may be detected at the left upper sternal border, reflecting severely elevated PVR.
Factors that can influence hemodynamics in Down syndrome include chronic nasopharyngeal obstruction, relative hypoventilation, carbon dioxide retention, and sleep apnea. Nonspecific CHF signs that may be seen include hepatosplenomegaly, pulmonary rales, and tachypnea. Skull erosion and striations have been noted from venous distension and increased blood volume.

Techniques of Operation
1. Direction 1) Closure of atrial communication 2) Closure of ventricular communication 3) Avoidance of damage to conduction 4) Creation of two competent valves 2. Technique 1) Repair of partial AV canal defect 2) Repair of complete AV canal defect one - patch technique two - patch technique 3) Repair of associated cardiac anomalies

Indications for Operation

1. Partial AV canal defect
Optimal age for operation is 1-2 years of age except when CHF or growth failure is evident earlier in life

2. Complete AV canal defect

Operation is indicated early in the 1st year of life when the infants general condition is good, repair can be delayed until 3-6 months of age.

3. Coexisting cardiac anomalies

Although certain major cardiac anomalies increase risk of AVSD, their presence rarely alters the indication for operation.

AV Valve Repair in AVSD

The most anterior point of LSL-LIL opposing edge should be found and sutured through it, and the anterior edges be sutured to the polyester patch The patch must be appropriate dimension & configuration and tailoring the waist of the patch is critical Remodeling leaflet closure by suturing portions of left superior leaflet and left inferior leaflet together in areas of regurgitation. Annuloplasty at commissure and making the edge of the pericardial patch along it shorter than the combined length of the base of leaflet

Surgical technique
Two techniques are widely used, a 1-patch technique and a 2-patch technique. First elevate the common AV valve to its closed position by injecting cold isotonic NaCl solution into the ventricles to assess valvular competence and structure. The central apposition of the SBL and IBL is the area where the 2 leaflets meet at a point separating the left and right AV valves. Identify and mark these points with fine polypropylene Sutures.

Surgical technique-cont.
Fashion a patch of polytetrafluoroethylene (PTFE, Gore-Tex) into a crescent shape to match the dimensions of the VSD. Secure this patch along the ventricular septal crest slightly on the rightward aspect, particularly inferiorly, to avoid the conduction system. For the 1-patch technique, divide the SBL and IBL along a line separating them into right and left components. Tailor a single polyethylene terephthalate (Dacron) or PTFE patch to close both the VSD and ASD

Surgical technique-cont.
Secure the patch to the crest of the ventricular septum. Then, resuspend the leaflets to the patch by passing interrupted sutures through the cut edge of the left AV valve leaflet, the patch, and the cut edge of the right AV valve, and tie the sutures closure of the cleft is an important mechanism in preventing postoperative left AV valve regurgitation Significant AV valve regurgitation, severe dysplasia of the left AV valve, and failure to close the cleft of the left AV valve as important risk factors for repeat surgery. Significant postoperative left AV valve regurgitation is also a ri sk factor for surgical and long-term mortality.

A. Single patch repair B. Modified single patch repair C. Double patch repair

Septal Patch for AVSD Repair

Too wide patch, theoretically left ventricular outflow obstruction & long patch with high AV valve level, possible AV valve regurgitation

Surgical technique-cont.

Closure of the cleft is an important mechanism in preventing postoperative left AV valve regurgitation Significant AV valve regurgitation, severe dysplasia of the left AV valve, and failure to close the cleft of the left AV valve as important risk factors for repeat surgery. Significant postoperative left AV valve regurgitation is also a risk factor for surgical and long-term mortality.

AVSD. Repair of Mitral Cleft

Partial Annular Plication

Two furling stitches with 3 pledgets or three furling stitches with 4 pledgets are placed along the annulus of either or both sides for mitral valve regurgitation

AV Valve Replacement
After AVSD Repair

Lengthening the mitral-aortic septum, thus the valve is well away from the LVOT

Features of Postoperative Care

1. Vigilance must be exercised to detect any important imperfections in the repair 2. LAP is higher 6 mmHg than CVP : suggest mitral valve stenosis or insufficiency 3. Prophylaxis against PA hypertensive crisis 4. Evaluation on left AV valve regurgitation : predispose patient to death within 1 year 5. Evaluation of left to right shunt 6. Reoperation is indicated in severe regurgitation and significant residual shunt

Postoperative details
Ventilator maneuvers include high FiO2, lowering of PCO2 (25-30 mm Hg), avoidance of acidosis, and use of inhaled nitric oxide (5-80 ppm). Some authors routinely use phenoxybenzamine (1 mg/kg) at the initiation and conclusion of cardiopulmonary bypass, as well as every 8 -12 hours postoperatively (0.5 mg/kg) in high-risk patients. Intravenous nitroglycerine, nitroprusside, aminophylline, and prostacycl in all have been advocated for the management of pulmonary hypertensive crises. Generally, avoid high-dose dopamine and alpha-adrenergic agents if possible. Carefully evaluate low cardiac output with TEE and, if necessary, cardiac catheterization.

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Complications
Most repeat surgeries following repair of AVSD are because of left AV valve regurgitation. Significant postoperative AV valve regurgitation occurs in 10-15% of patients, necessitating additional surgery for valve repair or replacement in 7-12% of patients. With improved understanding of the conduction system in AVSDs, incidence of permanent complete heart block is approximately 1%, as reported by Studer et al and Kadoba et al. Heart block encountered in the immediate postoperative period may be transient and result from edema of or trauma to the AV node or bundle of His. However, according to Kadoba et al, right bundle branch block is common (22%).
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Results of Operation
1.Survival 5) accessory valve orifice 1) early death 6) major associated cardiac 2) time related survival anomalies 2. Mode of death 7) young age ; not now 1) early : acute cardiac failure and 8) Down syndrome pulmonary dysfunction 9) need for reoperation 2) late : chronic or subacute 10) single papillary m. cardiac failure 11) hypoplasia of ventricle 3. Incremental risk factors for 4. Heart block & arrhythmia premature death 5. Functional class 1) earlier date of operation 6. AV valve function 2) functional class 7. LVOT obstruction 3) prerepair AV valve incompetence 8. Residual pulmonary 4) interventricular communication hypertension

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