Download Congenital Heart Diseases II

Congenital Heart Diseases
II
Samer Abbas, MD
Cardiology UIC/Christ
02/27/2008
Classification
Cyanosis with low
pulm flow:
- TOF
- Ebstien Anamoly
- Hypoplastic of RV
- Tricuspid atresia
- Pulm atresia
Cyanosis with high
pulm flow:
- TGA
- Double outlet V.
- Double inlet V
- TAPVD.
Eisenmenger Syndrome
In 1897 Victor Eisenmenger published
a paper entitled “Congenital Defects of
the Ventricular Septum.”
In 1958, Paul Wood summarized
Eisenmenger’s accounts:
“The patient was a powerfully built man of 32
who gave a history of cyanosis and moderate
breathlessness since infancy. He managed well
until January of 1894 when dyspnea increased
and edema set in. Seven months later he was
admitted to the hospital in a state of heart
failure……He improved with rest and digitalis,
but collapsed and died suddenly on November
13 following a large hemoptysis”
Eisenmenger Syndrome
Large left to right
shunt:
– severe pulmonary
vascular disease leading
to shunt reversal
Initial reversible
changes:
– Medial hypertrophy of the
pulmonary vasculature
– Intimal proliferation
Eisenmenger Syndrome
Progressive irreversible
changes:
– plexiform lesions
– necrotizing arteritis
As the increased PVR
approaches or exceeds the SVR
the shunt is reversed.
As R to L shunting develops
cyanosis appears.
Most patients will develop
exertional dyspnea and impaired
exercise tolerance.
Eisenmenger Syndrome
Palpitations occur in >50% of patients
– A. fib/flutter in 40%
– VT in 10%)
Hemoptysis in ~20%
PE, angina, syncope, endocarditis ~10%
Signs of PHTN
– RV heave, palpable P2, and right sided S4
Pulmonary ejection click and a soft scratchy
SEM
– d/t dilated pulmonary trunk
High pitched decrescendo diastolic murmur
(Graham-Steele) audible in most patients
Usually no peripheral edema until R HF ensues
Eisenmenger Syndrome
ECG shows RVH, RAE and RAD
Atrial arrhythmias may be present
Eisenmenger Syndrome
CXR reveals prominent central pulmonary arteries
and decreased vascular markings (pruning) of the
peripheral vessels
Eisenmenger Syndrome
Large variation in life expectancy in adults with
Eisenmenger syndrome
Rate of survival among patients with Eisenmenger
syndrome is:
– 80% at 10 years
– 77% at 15 years
– 42% at 25 years
Recent study of 109 adults revealed following as
independent predictors of mortality:
– Age at presentation
– Supraventricular arrhythmias
– Poor NYHA functional class (III or IV)
Eisenmenger Syndrome
Pregnancy is discouraged due to high maternal (50%) and
fetal (60%) mortality.
CVA may occur secondary to paradoxical emboli
Cerebral abscesses
Avoid intravascular volume depletion, heavy exertion, high
altitudes, and use of vasodilators
IV epoprostenol may be beneficial in decreasing PVR
Phlebotomy with isovolumic replacement is recommended
for patients with moderate to severe symptoms of
hyperviscosity and an elevated hematocrit >65%
Prevention of iron deficiency is important
Supplemental oxygen reduces episodes of dyspnea
Lung transplantation (with repair of cardiac defect) or
heart/lung transplantation is an option
Tetralogy of Fallot
Etienne-Louis Arthur
Fallot made the first
published bedside
diagnosis that was
proven at postmortem in 1888 and
called the condition
of ‘maladie bleue’.
Epidemiology
Prevalence varies from 0.26-0.48/1000 live births.
Exact incidence of TOF with PS versus pulmonary
atresia or absent pulmonary valve is not known as most
studies lump them together.
Most recent study is the BWIS (Baltimore –Washington
Infant Study)
-Incidence 0.33/1000 live births
-Fifth most common defect overall (6.8% of all CHD)
-Most common cyanotic CHD
-TOF with PS 0.26/1000 live births (5.4% of all CHD)
-Suggestion of male predominance ( p=NS)
Genetics
Syndromes associated with TOF:
–
–
–
–
–
–
DiGeorge’s
Velocardiofacial
Alagille’s
Trisomy 21/18/13
Cat Eye
San Luis Valley
Mutations of Nkx 2.5 may be associated with
TOF.
Morphology
Ventricular Septal Defect
– 80% of cases fibrous
continuation between the
mitral, tricuspid, and aortic
valves
– 20% cases there is a
muscular rim around the
defect.
– Rarely subarterial defect
due to hypoplasia of the
outlet septum.
– The pulmonary valve
annulus tends to be
hypoplastic or atretic.
Morphology
Overriding Aorta
– Degree of override can
vary from an exclusive
connection of the right
ventricle to an
exclusive connection
to the left ventricle.
Pulmonary Stenosis:
– Infundibular
– valvular
Morphology
Concentric right
ventricular
hypertrophy:
– Secondary to RV
outflow obstruction
Associated Lesions
Pulmonary valvar stenosis
Pulmonary atresia
Absence of pulmonary valve leaflets
– pulmonary insufficiency in utero and marked dilatation
of the pulmonary trunk.
Right aortic arch
Patent foramen ovale
Atrial Septal Defect ( Pentalogy of Fallot)
Anomalous origin of the LAD from the RCA.
On Exam
Cyanosis of varying degrees
Clubbing
Accentuated RV impulse
Second heart sound usually single and loud. If mild PS
with normal valve apparatus S2 normally split.
Pulse pressure may be wide in the presence of
aortopulmonary collaterals or PDA.
On Exam
Systolic Murmur:
– Upper left sternal border, crescendo-decrescendo or
plateau
– Intensity inversely proportional to severity of the right
to left shunt
– In PS with intact septum murmur directly proportional
to the severity of stenosis
– Murmur decreases in intensity during hypercyanotic
spells
Diastolic Murmur:
– If AI concomitantly heard in TOF with APV syndrome
Tetralogy of Fallot
Tetralogy of Fallot
Catheterization
Surgical repair
Closure of VSD
Relieving the RVOTO
–
–
–
–
Pulmonary valvotomy
Resection of infundibular muscle
RVOT or subannular patch
Transannular patch
only when PV annulus is restrictive
– Pulmonary valve implantation
usually in adolescents and adults
– Extracardiac conduit between the RV and PA
Angioplasty or patch augmentation of the central pulmonary
arteries
– Closure of ASD or PFO
Complications After Repair
Mechanical problems
Significant Pulmonary
regurgitation
RV Dilatation
Restrictive RV
Residual RVOTO
Aneurysmal dilatation
of the RVOT Residual
VSD
AR with or without
root dilatation
LV dysfunction
Infective endocarditis
Complications after repair
Electrical Problems
Heart block: RBBB, Bifascicular.Complete heart block is
rare.
Supraventricular arrythmia (A.flutter/A.fib)
Relatively common. A.flutter-SCD,1:1.
NSVT: Significance uncertain, ? SCD
VT
SCD
Risks for SCD/VT
Older age at repair
Male gender
Worse heart failure NYHA Class
Chronic RV volume overload
Presence of PR
QRS duration >180 msec
Transient complete heart block
lasting more than 3 days after
repair.
Management of the complications
Significant PR and RV dilatation
– Pulmonic valve replacement with homograft or
porcine bioprosthesis
– Timing of surgery is debated. Selection of
asymptomatic patient ?criteria.
– Might TV annuloplasty if concomitant moderate to
severe TR.
Restrictive RV
– Diuretics in the post-operative period.
Residual RVOTO
– Indicated if RVSP>2/3 systemic pressure.
– Surgery or balloon angioplasty with stenting.
Management of complications
Aneurysmal dilatation of the RVOT
Residual VSD:
– Surgical correction if Qp/Qs>2:1 or 1.5-2:1 with LV
dilatation or dysfunctionor H/O paradoxical emboli
Dilatation of aortic root:
– Aortic root replacement when ascending aorta>55
mm and/or in the presence severe AI.
Management of the Complications
Heart block:
– Patients with bifascicular block with prolonged PR
(>0.20 msec) should have holter or EPS. If true
trifascicular block is documented then pacemaker.
SVT
– Correction of target residual hemodynamic lesions.
– RF ablation for a.flutter or reentrant tachycardia either
percutaneously or during surgery.
– Biatrial maze for a.fib.
– Anti-arrhythmic meds /antitachycardia pacemakers
can be used as adjunctivetools.
Management of Complications
VT
–
–
–
–
Correction of the hemodynamic lesions
RF ablation of VT focus
Role of AICD (?)
Antiarrhythmic meds for symptomatic patients.
SCD
– Resuscitated SCD-AICD
– Role of prophylactic AICD (?)
Fontan procedure-requiring patient
Tricuspid atresia
Hypoplastic left ventricle
Double inlet ventricle
Isomerism
Tricuspid atresia
Tricuspid Atresia
On Echo
Hypoplastic Left Heart
Double Inlet Ventricle
Fontan procedure
10 year survival is 60-70%
Poor prognosis
– 5 year survival 46-59%
Fontan Patient
Long term complications include
– Atrial flutter or fibrillation
Incidence increases with time
– Right atrial thrombus formation
– Obstruction of the Fontan circuit
Should be r/o in all pts presenting with atrial arrhythmias
– Ventricular dysfunction
– Protein losing enteropathy
Etiology unclear: pleural effusions, ascites, edema and chronic
diarrhea. Low S.Albumin, high α-1 antitrypsin stool clearance.
Management of Complication
In patients with atrial fibrillation and flutter
– Prompt TEE followed by cardioversion
– Maintenance of sinus with drugs ( Amiodarone) <50%
success rate.
– Transcatheter atrial ablation <50% success rate
– Recalcitrant cases surgical revision with
antiarrhythmic surgery
Management of Complications
In patients with established thrombus
– Thrombolytic therapy
– Surgical thrombectomy
– Conversion of Fontan circuit
– Long term anticoagulation
– Many centers prophylactically anticoagulate all
patients with Fontan.
Management of complications
In patients with Fontan obstruction
– Surgical revision
– Balloon angioplasty
Protein losing enteropathy
–
–
–
–
Creation of a fenestration in the atrial septum
Heparin SQ
IV Octreotide
Prednisone
In ECHO
The presence or absence of right atrial stasis,
thrombus, patency of a fenestration, and Fontan
circuit obstruction should be sought.
Superior and inferior vena cavae biphasic and
pulmonary artery triphasic flow patterns suggest
unobstructed flow in the Fontan circuit.
Mean gradient between the Fontan circuit and
the pulmonary artery of 2 mm Hg or more may
represent significant obstruction.
Conduit or Lateral Tunnel Fontan
Fenestration Fontan
TGA
D loop
L loop
RV follow the Loop
– D loop------RV on the right
– L loop------RV on the left
L TGA
Congenitally
corrected TGA.
Ventricular inversion
D TGA
Incidence 20-30/1000,000 live births.
Without treatment 30% die within 1st week, 50% within
1st month, 70% in 6 months and 90% in first year.
With current medical and surgical interventions 90%
early and midterm survival.
In complete Transposition the connection between atria
and ventricles are normal.
The connections between the ventricles and the great
arteries are discordant.
D TGA
Surgery
Most commonly done procedures were atrial
switch operations:
– Blood redirected at atrial level with a baffle made of
dacron or pericardium (Mustard)
– With atrial flaps (Senning) to achieve physiological
correction
Surgery
Current practice is the arterial switch operation
developed in 1980’s.
Blood is redirected at the level of the great
artery
– the morphological left ventricle becomes the subaortic
ventricle
– the morphological right ventricle becomes the
subpulmonic ventricle.
Atrial Switch Surgery (Mustard/Senning
Procedure)
Mustard Procedure
Mustard Procedure
Modified Arterial Switch Operation
Consequences of Mustard
Most patients reaching adulthood have NYHA I/II
symptoms over the next 25 years.
50% develop moderate systolic dysfunction of the RV
but only few present with CHF.
1/3rd have severe systemic TR.
Atrial flutter arises in 20% by age 20.
50% patients have sinus node dysfunction by age 20.
Baffle leak or obstruction can also occur.
Baffle Leak
Consequences of Arterial Switch
Data about long term consequences still
unavailable.
Concerns include:
– supra neopulmonary artery stenosis
– ostial coronary artery disease
– progressive neoaortic valve regurgitation
Treatment
Use of ACE inhibitors suggests a benefit in nonrandomized studies.
– randomized study using Ramipril is underway.
Failed Mustard or Senning might be a reason for heart
transplantation.
Alternatively, atrial baffle can be converted to an arterial
switch after retraining the LV with a pulmonary band.
– No data available for outcomes
TV replacement is not recommended since it worsens RV
function.
Ebstein’s anomaly
May be associated with:
•PFO
•ASD
•Most commonly secundum
rarely primum
•VSD
•with or without pulmonary
atresia
•Pulmonary outflow
obstruction
•PDA
•Coarctation of the aorta
•LV cardiomyopathy
Ebstein’s Anomaly
Prevalence 1:50,000 to 100,000. Occurs with higher
frequency in infants of mothers who took Lithium in
early pregnancy.
Clinical Features
Depends on the severity of the pathology and the magnitude of right to
left intraatrial shunting.
In milder forms of the condition incidental murmur and arrhythmia may
be the only features.
In severe forms of the disease cyanosis is seen and depends on the
extent of right to left shunting.
Increased incidence of WPW syndrome.
Echo
Increase in RV volume
Paradoxical septal motion
Increase in TV excursion
Delay in TV closure
compared to MV closure
Decreased TV EF slope
Abnormal anterior
position of the TV in
diastole
Prognosis and Management
Survival at 1 year 67% and 10 year 59 %.
Survival after Tricuspid bioprosthesis: 10 yr 93%
and 15 yr 81%. 94% in NYHA I/II.
Management consists of surgical repair of the
TV or replacement in older individuals.
Pregnancy
BP decrease early in pregnancy ~10 mmHg,
usually secondary to decreased SVR
This increases flow across right to left shunts.
30-50% increase in intravascular volume. Poorly
tolerated by women with valvular lesions and
LV/RV dysfunction.
Marked fluctuations occur in CO during normal
labor and delivery.
Pregnancy
Major maternal cardiac risk factors
Pulmonary HTN
-Maternal mortality 30-50% with Eisenmenger syndrome.
Maternal Cyanosis
-Cyanotic worse then acyanotic.
-Erythrocytosis worsens outcome.
-Goal Hct<65
Poor maternal functional class
-Difficult to assess in cyanotic CHD.
H/O Arrhythmia
-Antiarrhythmic drugs can be used with accepted risk for debilitating
symptoms
Anticoagulation
Specific Patients
TOF
– Uncorrected TOF associated with worsening cyanosis.
Susceptible to Infective endocarditis.
– Corrected TOF usually uncomplicated pregnancy
Ebstein’s Anomaly
-Worsening RV function in pregnancy
-Atrial tachyarrhythmias/ WPW
-Cyanosis might be manifest for the first time
-Increased risk of paradoxical embolization and hypoxemia
-Acyanotic women with Ebstein’s tolerate pregnancy well if
sinus rhythm is maintained.
-Repaired Ebstein’s significantly reduces risks
Specific patients
Congenitally corrected TGA (L-TGA)
-Increased risk of ventricular volume overload
-Increased risk of heart block
Complete TGA
-Atrial baffle- Risks are related to functional status of the
RV, PAP and rhythm disturbances.
-Not enough experience with arterial switch.
Fontan
-Usually leads to uncomplicated pregnancy.
-Risk of thrombus formation.
Labor and delivery
Women with unoperated or operated CHD who are functionally
normal can be allowed to progress to spontaneous labor
If functional adequacy of heart and lungs in doubt:
-Controlled induction
-Oxytocin low dose for induction.
-Avoid maternal pushing
-Assisted delivery with low forceps or vacuum extraction.
Anesthesia
-IM/IV narcotics safe.
-Epidural safe to use :caution systemic venous pooling and
increased thromboembolism risk.
Labor and delivery
No indication for routine Swan Ganz
Routine endocarditis prophylaxis
Cesarean delivery reserved for obstetric indications
-A/W twice the amount of blood loss
-Increased risks of wound and uterine infection
-Increased incidence of post-op thrombophlebitis.
Breastfeeding
-Affects cardiac reserve and risks mastitis and
bacteremia.
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