left-right shuntnatural history & principles of management
TRANSCRIPT
Most common congenital heart defect in children
Incidence is 8 per 1000 live births
Echo studies- 5 to 50 per 1,000 newborns
Ooshima A et al. Cardiology 1995;86:402-406.
No sex preference , except in subarterial defect
ASIAN WESTERN
Doubly commitedsubarterial
Multiple ventricular septal defects are rare
Doubly-commitedsubarterial defect requiring repair is 30%
Muscular defects
10% in the west
5% in western
Ferreira Martins JD et al.Cardiol Young 2000; 10: 464–73.
Pathologic anatomy of IVS
Soto et al classification of VSD Perimembranous (membranous/ infracristal )-70-80%
Muscular- 5-20% Central- mid muscular
Apical
Marginal- along RV septal junction
Swiss cheese septum – multiple defects
Inlet/ AV canal type-5-8%
Supracrital (Conal/ infundibular/subpulmonary/doubly committed subarterial)- 5-7%
Benigno soto et al. Br HeartJ 1980; 43: 332-343
Restrictive- less than one third of aortic root LVSP > > RVSP
Pulm /Aortic systolic pressure ratio < 0.3
Qp / Qs<1.4:1
Moderately restrictive RVSP high, but less than LVSP
Qp/Qs 1.4:2.2
Non restrictive - The size of aortic orifice RVSP , LVSP, PA Aortic systolic pressures are equal
Qp/Qs >2.2
Flow determined by PVR
Anterior more common than posterior
Usually involves the infundibular septum
Occurs as if there is a door that moves round on a hinge
The outcome and natural history influenced by
Position & Size
Number of defects
Anatomic structures in the vicinity of the defect
Association of other malformation
Age at which the defect is recognized
Sex of the patient
Cardiac failure
Spontaneous diminution in size or closure
Right or Left ventricular outflow tract obstruction
Aortic regurgitation
Pulmonary vascular obstructive disease
Infective endocarditis
Rare in small VSD as size limits the L-R shunt
In large VSD the relative resistances of the systemic and pulmonary circulations regulate flow
Shunt occurs mainly in systole
Shunt directly to PA
Enlargement of LA, LV,PA
After birth decline in PVR to adult level by 7to 10 days
In large VSDs, the rate of this process is delayed
Small VSD the shunt is small & remain asymptomatic
Moderate sized VSD symptoms by 1to 6 months
Rarely, adults present with new exercise intolerance
.)
Rudolph AM, et al.Pediatrics 1965;36:763-772.
Large VSD congestive heart failure in first few weeks
Risk for recurrent pulmonary infection high
If survives without therapy - pulmonary vascular disease develop in the first few years of life
Symptoms “get better” as Qp/Qs returns to 1:1
Intervention at this time - a shorter life expectancy than if the defect were left open
Fuster V, et al.Cardiovasc Clin. 1980;10:161–197.
Occurs in both perimembranous and muscular
Closure is documented in the fetus & in the adult
VSD diagnosed in the fetus - 46% closed in utero & 23.1% in the first year, while 30.8% remained patent
In this study 15.8% of defects < 3 mm remained patent in comparison to 71.4% > 3mm at 1yr
Nir A et al.Pediatr Cardiol 1990; 11: 208–10.
More frequent in <10 yrs of age
Isolated VSD ( 124 pts) - 34% at 1 yr & 67% at 5 yr
Female predominance
Decreases substantially after 1 year of age
Mehta AV et al. Tenn Med 2000; 93: 136–8.
Farina MA et al . J Pediatr 1978; 93: 1065–6.
Moe DG et al. Am J Cardiol 1987; 60: 674–8.
Rare in malaligned VSD
In outlet VSD closure only in 4%
All of the defects closed were initially < 4 mm
Tomita H et al. Jpn Circ J 2001; 65: 364–6
Different for perimembranous and muscular
Perimembranous Reduplication of tricuspid valve tissue
Progressive adherence of the septal leaflet of the tricuspid valve about the margins of the VSD
Aneurysmal transformation of the membranous septum (appearance on angiography)
Early systolic click & late crescendo systolic murmur
Anderson RH et al . Am J Cardiol 1983; 52: 341–5.
Freedom et al. Circulation 1974; 49: 375–84.
Muscular- direct apposition of muscular borders
Large subarterial defect don’t close
A. Closure of a perimembranous defect by adhesion of the tricuspid leaflets to the defect margin.
B. Closure of a small muscular defect by a fibrous tissue plug.
C. Closure of a muscular defect by hypertrophied muscle bundles in the right ventricle
D. Closure of a defect in subaortic location by adhesion of the prolapsed aortic valve cusp
Incidence 3% to 7%.
Mechanism:- Hypertrophy of malaligned infundibular septum
Hypertrophy of right ventricular muscle bundles
Prolapsing aortic valve leaflet
High incidence inRight sided aortic arch
Horizontal RVOT
Nadas AS et al . Circulation 1977; 56(No.2, Suppl. I): 1–87.
Corone P et al. Circulation 1977; 55: 908–15.
Pongiglione G et al . Am J Cardiol 1982; 50: 776–80.
Varghese PJ et al . Br Heart J 1970; 32: 537–46.
Tyrrell MJ et al. Circulation 1970; 41 & 42(Suppl. III): 113.
VSD with direct contact with the aortic valve are most prone to develop AVP
All the perimembranous defects
All doubly committed juxtaarterial defects
Most of muscular outlet defects
Characteristic deformity of aortic cusp-nadir of the cusp is elongated
RCC (60-70%) , NCC (10-15%) , both in 10-20%
Non-coronary cusp prolapse in perimembranous type
Left coronary cusp prolapse extremely rare
AR may be due to incompetent bicuspid aortic valve
Rarely prolapsed valve cusp may perforate
Pathogenesis
Anatomic factors for normal competence Leaflet support by diastolic apposition
Infundibular support from below
Intrinsic structural abnormalityProgressive discontinuity between aortic valve annulus & media
PathogenesisHemodynamic factor
‘’Venturi effect’’
VSD is restrictive, Qp/Qs<2 , absence of PAHKomai H et al.Ann Thorac Surg 64:1146-1149, 1997
Unknown exact prevalence (2% to 7%)
Rare before 2 years
More severe - additional volume load
Aneurysm of sinuses of Valsalva may develop
Nadas AS et al . Circulation 1977; 56(No.2, Suppl. I): 1–87.
362 Patients. 37 (10.2%) had AR
Mean age 13.4 years ( 2-45),male to female ratio 5:1
31 (84%) had infracristal & 6 (16%) supracristal VSD
Infracristal VSD-RCC prolapse in 14 (48%) &NCC in 12 (41%) and both RCC and NCC in 3 (11%)
Supracristal VSD - RCC prolapse in 5 (83%),NCC in 1
Two patients the AR was due to bicuspid aortic valve
PA pressure normal in 26(70.2%), L-R shunt 1.5:1 or less in 23 (62%)
No relationship- severity of AR & location of VSD
Somanath HS et al. Indian Heart J. 1990 Mar-Apr;42(2):113-6.
Indicated for both perimembranous and subarterial VSDs when more than trivial AR
Subarterial VSDs >5 mm - closed regardless of AVP
Restrictive perimembranous VSD with AVP but without AI, surgery indications are less clear
Follow up regularly
Surgery is indicated only if AI develops
Elgamal MA et al . Ann Thorac Surg 68:1350-1355, 1999
Lun K et al. Am J Cardiol 87:1266-1270, 2001
Gabriel HM et al. J Am Coll Cardiol 39:1066-1071, 2002
Usually above the ventricular septal defect
Etiology:-
Progression of the pre-existing lesion
Acquired
Two types:-
Muscular
Fibromuscular
Three major structures are responsible
Posteriorly malaligned outlet septum
Septal deviation ( anteroseptal twist)Muscular protrusion of the left ventricular aspect of the septum
Anterolateral muscle bundleMuscular protrusion between LCC & AML- present normally in 40%
(Very rarely “Mitral arcade”)
Careful echo assessment of the LVOT should be performed in all cases of VSD before surgery
Moene RJ et al. Pediatr Cardiol 1982;2: 107–14.
Incidence - 5% to 22%
Rare in small & Moderate-size VSDs
Down syndrome – early development of PAH
No overall sex predilection
Keith JD et al. Heart Disease in Infancy and Childhood. 1978: 320–79.
Survival rate for patients with VSD by pulmonary artery systolic pressure
42 men and 37 women, 18 to 59 years (mean 34 yrs)
67 patients treated medically and 12 surgically
All patients were followed up for 1 month to 25 yrs ( mean 9yrs)
The solid line indicates a pressure less than 50 mm Hg (n = 36)
Dashed line indicates a pressure of 50 mm Hg or greater (n = 17)
Ellis JH 4th et al . Am Heart J. 1987;114:115-205
Eisenmenger complex, develops in 10% to 15%most commonly in the 2 nd & 3rddecades of life
common causes of death "sudden" or "unknown“
Development of pulmonary vascular disease after surgery depends on age at procedure is done
Infants with VSD and increased pulmonary artery pressure - repair between 3 and 12 months
18.7 per 10000 person-years in non operated cases
Operated VSD 7.3 per 10000 person-years
Higher in small defect & lower during childhood
Patients with a proven episode of endocarditis are considered at increased risk for recurrent infection so surgical closure may be recommended
Gersony WM et al.Circulation 1993; 87(Suppl. I):I-121–I-126.
Patients with VSD have a high incidence of arrhythmia
Ventricular tachycardias in 5.7%
Sudden death is 4.0%
SVT, mostly AF, is also prevalent
Age and pulmonary artery pressure are the best predictors of arrhythmias
The odds ratio of serious arrhythmias increases
1.51 for every 10-year increase in age
1.49 for 10mm Hg increase in mean PA pressure
Wolfe RR et al. Circulation. 1993;87:I89-101
Closing defect - soft S2, high frequency & shorter murmur
Increasing PVR : increased RV pulsations ,S2 loud & narrow split
Infundibular hypertrophy & resulting decreased L to R shunt : S2 decreases in intensity ,crescendo-decrescendo systolic murmur in the ULSB
Heart failure not controlled by medical therapy VSD should be operated with in 6 month of life
Qp/Qs is 2 or more surgical closure needed regardless of PA pressure
VSD with PVR more than 4 unit during 6-12 months
VSD with elevated PVR first seen after infancy
Moderate VSD with no size change in childhood
Right bundle branch block 33.3% undergoing transatrial repair
78.9% to 11% in repair via a right ventricular incision
Transpulmonary approach has the lowest incidence
Complete heart block in 1 to 2%
Pulmonary hypertension in (4% )
Sinus node dysfunction (4%)
Progressive aortic valve insufficiency (16%)Roos-Hesselin JW et al. Eur Heart J 2004;25:1057-1062.
Abe T et al . Jpn Circ J 1983; 47: 328–35.
Patent Ductus Arteriosus
Incidence of isolated PDA in term infants - 1 in 2,000
Female predominance - 3:1
High incidence- Prematurity, Maternal rubella
Genetic inheritance- Autosomal recessive with incomplete penetrance
Congestive heart failure
Infective endarteritis
Pulmonary vascular disease
Aneurysmal formation
Thromboembolism
Calcification
CHF resulting from an isolated PDA either develops in infancy or during adult life
HF in infancy usually occurs before of 3 mths of age
Initially left heart failure, later right heart failure
Good response to drugs initially, but is not maintained
Major cause of death in earlier era
Incidence - 0.45% to 1.0% per annum
Vegetations usually found at the PA end of the duct
May cause recurrent pulmonary embolism
Infection may also cause a ductal aneurysm
Cosh JA. Br Heart J 1957; 19: 13–22.
No definite data on incidence
“Differential cyanosis”
Eisenmenger patients do not tolerate PDA closure
Described either pre- or postnatally
Likely develops in the third trimester due to abnormal intimal cushion formation or elastin expression
Incidence varies from 1.5% to 8.8%
Dyamenahalli U et al. J Am Coll Cardiol 2000; 36: 262–9.
Jan SL et al. J Am Coll Cardiol 2002; 16: 342–7.
Complications
Thromboembolism
Dissection
Rupture
Tracheal compression
Left recurrent laryngeal nerve palsy
Pulmonary artery obstruction
Regression can occur, presumably due to thrombosis and subsequent organization
As many ducts will eventually close in premature infants approach is different - preterm infant Vs mature, child
Beyond infancy, closure reported in 0.6% per year
Medical therapy Treatment of Heart failure
Ductal closure with drugs
Surgical Therapy
Campbell M et al.Heart 1968;30:4–13.
Indomethacin Rates of success varying between 18% to 89%
Major determinants of success were gestational and postnatal age
Less chance of closure-Extreme prematurity, very low birth weight, and advanced postnatal age
The rate of re-opening is highest in the extreme premature( 1/3) & less than 1/10 those weighing 1500 g
Mahony et al. N Engl J Med 1982;306: 506-510.
Ibuprofen
Comparable rate of ductal closure to Indomethacin
Less effect on renal function
Increased incidence of Pulmonary hypertension
Gournay V et al. Lancet 2004;364:1939-1944.
Coils closure for <3 mm, >97% success, zero mortality
Larger PDAs - specialized devices
>98% complete closure rate at 6 months
Symptomatic moderate or large PDA with L to R shunt
Prior history endarteritis [class1]
Reasonable in small L to R shunt with normal-sized heart chambers when the PDA is audible by auscultation[IIa]
May be considered in bidirectional PDA shunt due to PAH and obstructive pulmonary vascular disease but reversible to pure L to R shunting with pulmonary vasodilators [IIb]
Incidence is 1 child per 1,500 live births
More frequent in females than males by about 2:1
Most ASDs occur sporadically; however, a few families have the defect as a genetic abnormality.
Mutation in-TBX5, NKX2.5 , GATA4, Myosin heavy chain 6
Samanek M et al. Pediatr Cardiol1999;20:411–7
The natural history depends on
Size of the defect
Rt. & Lt. ventricular diastolic compliance
Pulmonary-to-systemic vascular resistance
Hemodynamic/anatomic abnormalities resulting from a secundum atrial septal defect include
Right ventricular and atrial volume overload
Pulmonary vascular obstructive disease
Tricuspid valve and/or pulmonary valve regurgitation
Supraventricular tachyarrhythmias
Shunt direction & magnitude are variable and age dependent
In fetal life, RV noncompliance, a result of high pulmonary vascular resistance, allows nearly unidirectional right-to-left flow at the atrial level
Immediately after birth, with RV compliance comparable to that of the LV, there may be little net shunting through ASD
With the physiological fall in pulmonary vascular resistance, the RV thins, compliance increases, left-to-right shunt develops
With similarly sized ASDs, adults have larger shunts
.)
Four common clinical presentations of ASD in adult
Progressive shortness of breath with exertion
Pulmonary vascular obstructive disease
Atrial arrhythmia
stroke or other systemic ischemic event
Most infants with ASDs are asymptomatic
They may present at 6 to 8 weeks of age with a soft systolic ejection murmur and possibly a fixed and widely split S2
CHF rare in the first decades of life but it can become common once the patient is older than 40 yrs
Atrial arrhythmia may be the first presenting sign (13% in older than 40 & 52% in older than 60 yrs of age)
Prevention of Atrial arrhythmia is one of the reasons for repairing ASD in young asymptomatic patients
(Silversides CK et al. Heart. 2004;90:1194 –1198.)
Subsequent development of AF may depend more on the patient’s age at intervention and may occur despite surgery in patients > 25 years of age
St. John Sutton MG,et al. Circulation 1981;64:402-409.
Murphy JG, et al. N Engl J Med. 1990;323:1645–1650
Uncommon in ASD
Incidence is 5% to 10% of untreated ASDs
Predominantly in females
Sinus venosus ASDs have higher pulmonary artery pressures & resistances than patients with secundum
Vogel M et al. Heart 1999; 82: 30–3.
Craig and Selzer in 1968 studied 128 adult patients
Significant PAH developed in 22% of the series
This complication usually develops when the patient is between 20 and 40 years of age
Cherian et al studied 709 pts.of isolated ASD
PASP was > 50 mmHg in 17%
PAH was present in 13% of patients under 10 yrs
14% of those aged 11 to 20 years
Eisenmenger syndrome 9%
Craig RJ et al. Circulation 1968; 37: 805–15.
Cherian G et al. Am Heart J 1983; 105: 952–7.
Spontaneous closure most likely in
ASDs <7 to 8 mm
Younger age at diagnosis
A review of 101 infants -mean age of diagnosis 26 days average follow-up of 9 months.
Spontaneous closure in all 32 ASDs <3 mm
87% of 3- to 5-mm ASDs
80% of 5- to 8-mm ASDs
None of 4 infants with defects >8 mmRadzik D, et al. J Am Coll Cardiol. 1993;22:851-853.
A follow up of 84 children for 4 years showed a spontaneous closure or decreased size in
89% with a 4-mm ASD
79% with a 5- to 6-mm defect
7% with a defect >6 mm
Helgason H, et al. Pediatr Cardiol.1999;20:195-199.
Even infants with CHF can have spontaneous reduction in the size of the ASD years after the diagnosis
Occasionally, spontaneous closure will occur as late as 16 years.
Evidence of Rt. sided cardiac volume loading, Qp:Qs > 1.5:1
Symptomatic patients (principally exercise related)
PVR < 7 WU – closure is usually well tolerated Need to be mindful of elevated LVEPD
Pts may need diuretic therapy after closure
For PVR >7 WU and PA pressures >50% need to perform O2 and NO study
Elective repair frequently has been deferred until the child is at least 4 years of age.
Early operation has been recommended for those infants and young children who have unremitting heart failure or associated pulmonary hypertension
contraindication :- pulmonary hypertension with Rt. to Lt shunting at rest
Pulmonary vascular resistance of 14 WU
1. In large VSDs, the rate of decline in pulmonary vascular resistance after birth is delayed
2. In Down syndrome the development of CHF is delayed
3. CHF symptoms get worsen initially as pulmonary vascular resistance increases
4. Adults may present with new symptom of exercise intolerance
1. Usually moderate sized VSDs can be managed medically without surgical intervention during infancy
2. Intervention to close the VSD with PAH would shorter life expectancy than if the defect were left open
3. There is enlargement of LA, LV, RV and PA in moderate sized VSDs
4. Development of PAH may occur in as many as a quarter of patients with large defects who undergo surgery after 2 years of age.
1. In outlet VSD 25 % will close by 2yrs
2. Isolated VSD closure rate - 34% at 1 yr & 67% at 5 yr
3. Closure is rare in malaligned VSD
4. Closure more frequent in <10 yrs of age
1. Rare before 2 years
2. Prevalence 2% to 7%
3. Right coronary cusp prolapse extremely rare
4. Non-coronary cusp prolapse in perimembranous type
1. Posteriorly malaligned outlet septum
2. Septal deviation or anteroseptal twist
3. Anterolateral muscle bundle
4. Mitral arcade
1. Incidence higher in large defect
2. Incidence lower during childhood
3. Operated VSD 7.3 per 10000 person-years
4. Surgical closure may be recommended in Patients with a proven episode of endocarditis
1. 2 days
2. 2 weeks
3. 2 months
4. 6 months
5. 12 months
1. Anterolateral muscle bundle present normally in 60% of infants
2. Anteriorly malaligned outlet septum is a major cause
3. Usually occurs below the ventricular septal defect
4. Septal deviation is a muscular protrusion of the left ventricular aspect of the septum
1. It is a shunt from the pulmonary artery to the aorta in the fetus
2. Blood flow may take place from the aorta to the pulmonary artery after birth, prior its closing
3. Right to left blood flow increases with the first breath after birth due to falling pulmonary vascular resistance
4. The smooth muscle in its walls is sensitive to rising PO2
1. Breathlessness
2. Failure to thrive
3. Asymptomatic murmur
1. Recurrent chest infections
1. Arrhythmias
1. In large VSDs, the rate of decline in pulmonary vascular resistance after birth is delayed
2. In Down syndrome the development of CHF is delayed
3. CHF symptoms get worse initially as pulmonary vascular resistance increases
4. Adults may present with new symptom of exercise intolerance
1. Usually moderate sized VSDs can be managed medically without surgical intervention during infancy
2. Intervention to close the VSD with PAH would shorter life expectancy than if the defect were left open
3. There is enlargement of LA, LV, RV and PA in moderate sized VSDs.
4. Development of PAH may occur in as many as a quarter of patients with large defects who undergo surgery after 2 years of age.
1. In outlet VSD 25 % will close by 2yrs
2. Isolated VSD closure rate - 34% at 1 yr & 67% at 5 yr
3. Closure is rare in malaligned VSD
4. Closure more frequent in <10 yrs of age
1. Rare before 2 years
2. Prevalence 2% to 7%
3. Right coronary cusp prolapse extremely rare
4. Non-coronary cusp prolapse in perimembranous type
1. Posteriorly malaligned outlet septum
2. Septal deviation or anteroseptal twist
3. Anterolateral muscle bundle
4. Mitral arcade
1. Incidence higher in large defect
2. Incidence lower during childhood
3. Operated VSD 7.3 per 10000 person-years
4. Surgical closure may be recommended in Patients with a proven episode of endocarditis
1. 2 days
2. 2 weeks
3. 2 months
4. 6 months
5. 12 months
1. Anterolateral muscle bundle present normally in 60% of infants
2. Anteriorly malaligned outlet septum is a major cause
3. Usually occurs below the ventricular septal defect
4. Septal deviation is a muscular protrusion of the left ventricular aspect of the septum
1. It is a shunt from the pulmonary artery to the aorta in the fetus
2. Blood flow may take place from the aorta to the pulmonary artery after birth, prior its closing
3. Right to left blood flow increases with the first breath after birth due to falling pulmonary vascular resistance
4. The smooth muscle in its walls is sensitive to rising PO2
1. Breathlessness
2. Failure to thrive
3. Asymptomatic murmur
1. Recurrent chest infections
1. Arrhythmias