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CUPRINS: Embriologie Clasificare Tablou clinic Diagnostic Tratament Prof univ dr Ion C.Tintoiu FESC
INCIDENCE
7 to 10 per 1000 live births Premature infants 2-3X higher incidence 2Most common form of congenital disease :ASD,VSD Accounts for 30% of total incidence of all congenital diseases 10% -15% have associated congenital anomalies of skeletal, RT, GUT or GIT Only 15% survive to adulthood without treatment
Embryologic Development
Braunwauld 6th ed
ETIOLOGY
10% associated with chromosomal abnormalities Two thirds of these occur with Trisomy 21 One third occur with karyotypic abnormalities such as Trisomy 13, Trisomy 18 & Turner Syndrome Remaining 90% are multifactorial in origin Interaction of several genes with or without external factors such as rubella, ethanol abuse, lithium and maternal diabetes mellitus
FETAL CIRCULATION
There are 4 shunts in fetal circulation: placenta, ductus venosus, foramen ovale, and ductus arteriosus In adult, gas exchange occurs in lungs. In fetus, the placenta provides the exchange of gases and nutrients
NORMAL HEMODYNAMIC
CHD CLASIFICARE
CLASSIFICATION OF CHD
CLASSIFICATION OF CHD
L - R SHUNTS INCLUDE :
ASD VSD PDA
AORTOPULMONARY WINDOW
ASD - Clinical
Symptomatic
Auscultation in ASD
Increased flow across the pulmonary valve produces a systolic ejection murmur and fixed splitting of the second heart sound. Fixed splitting of S2 may in part be due to delayed right bundle conduction. Increased flow across the TV produces a diastolic rumble at the mid to lower right sternal border.
Older pt loses pulm ejection murmur as shunt becomes bidirectional signs of pulm HTN/ CHF may predominate
Enlargement of the right ventricle Enlargement of atrium Large pulmonary artery increased pulmonary vascularity is.
ASD: Therapy
Percutaneous Closure
only for secundum (contra in others) adequate superior/inferior rim around ASD no R-L shunting RGood prognosis:
closure age < 25, PA pressure <40 If >25 or PA>40, decreased survival due to CHF, stroke, and afib
Surgical Closure
CORTRIATRIATUM
COR TRIATIATUM
VSD
Defined: A congenital abnormality in which blood flows from high pressure LV low pressure RV through a hole T- holosystolic L- 3rd, 4th, 5th left interspaces C- harsh, loud A- alone or with other abnormalities B- infant: late cyanosis, holo/pancystolic adult: progressive pulmonary HTN, Eisengengers syndrome
VSD
Large VSD: The presence of right ventricular hypertrophy, olegeimic lung fields (pulmonary hypertension or an associated pulmonic stenosis), gross cardiomegaly with prominence of both ventricles, the left atrium.
murmur
Wide pulse pressure Enlarged heart Thrill in L second IS Continuous murmur X-ray: prominent pulmonary artery with increased vascular markings.
OBSTRUCTIVE LESIONS
COARCTATION OF AORTA
HTN murmur (continuous or systolic murmur heard in back or SEM/ejection click of bicuspid AV)
Rib notching
Coarctation of Aorta
Narrowing in proximal descending aorta May be long/tubular but most commonly discrete ridge Natural hx: poor prognosis if unrepaired
Coarcation: Treatment
Despite surgery, patients still have significant morbidity/mortality with average age 38 Up to 70% of repaired patients still go on to develop HTN, pathology not well understood Recurrence in 8-54% of repairs, can undergo repeat 8surgery or balloon angioplasty Aortic Aneurysm/ruputure may occur despite successful repair and correction of HTN (freq around anastomosis site on patch repair 30% in one study)
Coarctation Repair
Surgical correction
1) Patch aortoplasty with removal of segment and end to end anastomosis or subclavian flap repair 2) bypass tube grafting around segment
Coarctation: Followup
Every 1-2 years 1Document arm/leg BP Screen/treat CAD risk factors HTN: rest, provoked by exercise or seen on ambulatory monitoring ECHO/doppler to eval recurrent MRI for aneurysm
AORTIC STENOSIS
Aortic Stenosis
Defined: Narrowing of the aortic outflow tract causing obstruction of flow from the left ventricle into the ascending aorta T- mid-systolic mid
L- apex- R 2nd intercostal space, radiates to carotids apexC- harsh, loud, may have associated thrill, ejection
click A- older age, bicuspid aortic valve, rheumatic fever B- age of patient, pulsus parvus et tardus, angina, syncope, heart failure
CYANOTIC CONGENITAL HD
TETRALOGY OF FALLOT
Tetralogy of Fallot
Tetralogy of Fallot
4 features
Malalignment VSD Overriding Aorta Pulmonic Stenosis RVH
Tetralogy: Treatment/complications
SystemicSystemic-Pulm shunt
leads to high flow through PA, elevated PVR and branch PA distortion survival after repair worse in pt with prior Waterston or Potts shunt (?higher flow); some pt with BlalockBlalockTaussig shunts may survive unrepaired into adulthood these pt should be evaluated for pulm artery stenosis and Pulm HTN
BlalockBlalock-Taussig Waterston (RPA) Potts (LPA) takedown of prior shunt patch VSD resection of subpulmonic obstruction transannular patch around pulm valve annulus (usually leads to severe PI)
Complete Repair
Tetralogy: Treatment/complications
Prior pulmonary valve atresia or anomalous LAD may have had prosthetic or homograft conduit valve placed between RV and PA Conduits can undergo endothelial overgrowth and obstruction of pseudo RVOT can Rx with balloon angioplasty or operative conduit replacement
Tetralogy: Risk/followup
risk can occur 2 decades after correction related to QRS duration> 180msec ? Due to PI/RV conduction defect atrial arrhythmias also common Chronic RV volume overload, RV dysfunction and exercise intolerance Pulmonic Valve Replacement can decrease QRS duration and stabilize RV fxn; timing unclear but earlier better than later RV fxn: ECHO or MRI
Hemodynamic effects of PI
Transposition D-type D PA arises from LV, Aorta from RV and anterior/right of PA cyanosis corrected initially with prostaglandin to keep ductus open and balloon atrial septostomy to improve systemic saturation repair via atrial switch Mustard procedure SVC/IVC baffled to LA/LV/PA Pulm Veins baffled to RA/RV/Ao Symptom free survival until 2nd-3rd decade of life repair via arterial switch long term data ? pulmonic valve (neo-aortic valve) competence?, reimplanted coronaries may develop ostial stenoses
D-Transposition complications
Complications
arrhythmias/SCD
Only 18% maintain SR; most go on to SSS/Afib/ Aflutter; pacemaker often needed ? TVR 15% have CHF sxs by 2nd-3rd decade Rx transplant or staged Arterial switch (pulm banding to train LV) Rare (5%) but serious complication; venous more common Suspect if new upper extremity edema (venous) or new CHF sxs (pulm venous) ECHO or Cath to eval, pulm venous obstruction Rx with surgery, systemic venous with angioplasty/stents
baffle obstruction
L-type Transposition
AtrialAtrial-ventricular AND ventricularventricular-arterial discordance Physiologically correct, anatomically incorrect congenitally corrected transposition RV is systemic ventricle, TV is systemic AV valve Asymptomatic for many years, often into adulthood
Although seemingly benign, survival is reduced with one study showing 25% of patients died by mean age 38 Progressive Heart Failure Arrhythmias
SCD AV block Atrial arrhythmias difficult to image using conventional ECHO MRI becoming test of choice for RV function
TRUNCUS ARTERIOSUS
EBSTEIN ANOMALY
Ebsteins Anomaly
Atrialization of RV, sail-like TV, sailTR 50% ASD/PFO 50% ECG evidence of WPW Age at presentation varies from childhood adulthood and depends on factors such as severity of TR, Pulm Vascular resistance in newborn, and associated abnormalities such as ASD
www.ucch.org
Pediatric
exercise intolerance
PULMONARY HYPERTENSION
EISENMENGER SYNDROM
Prominent pulmonary artery. Prominent right ventricle Prominent vascularity in the hilar areas Decreased vascualr marking in the periphery. No treatment
Rx
Eisenmengers Syndrome
Final common pathway for all significant L R shunting in which unrestricted pulmonary blood flow leads to pulmonary vaso-occlusive disease vaso(PVOD); R L shunting/cyanosis devleops Generally need Qp:Qs >2:1
Eisenmenger: Treatment
RULE OUT CORRECTABLE DISEASE Once diagnosis established, avoid aggressive testing as many patients die during cardiovascular procedures Diuretics prn, oxygen Definitive: Heart Lung transplant
Eisenmenger Complications
Coarcation: Treatment
Despite surgery, patients still have significant morbidity/mortality with average age 38 Up to 70% of repaired patients still go on to develop HTN, pathology not well understood Recurrence in 8-54% of repairs, can undergo repeat 8surgery or balloon angioplasty Aortic Aneurysm/ruputure may occur despite successful repair and correction of HTN (freq around anastomosis site on patch repair 30% in one study)
Coarctation: Followup
Every 1-2 years 1Document arm/leg BP Screen/treat CAD risk factors HTN: rest, provoked by exercise or seen on ambulatory monitoring ECHO/doppler to eval recurrent MRI for aneurysm
Palliative Surgery
RVOT reconstruction
Valvotomy Patch widening Valved conduit
Complex anomaly with severe cyanosis, irritability, hypoxic episode Critically ill neonates or infants due to decreased pulmonary f Facilitating growth of hypoplastic pulmonary artery
Atrial Septectomy
For the increasing of effective pulmonary flow and systemic oxygen saturation
Indication of atrial septectomy : TGA Tricuspid atresia Pulmonary atresia + IVS MV and LV hypoplasia Decreasing tendency of indication due to total correction or intervention
early
Reparative Surgery
NonNon-open heart surgery Open heart surgery Palliative procedure Corrective procedure
Significant PDA : indicated after 1st month Prophylactic closure : 6-12 mo 6Sx of heart failure or failure to thrive : indicated at any time Severe pulmonary vascular disease : contraindicated
Symptomatic large VSD : an indication of operation Before 3 mo : indicated in large VSDs with CHF or respiratory symptoms Moderate sized VSDs (Qp/Qs < 3.0) with few symptoms : observation during infancy Small VSDs (Qp/Qs < 1.5) : not indicated, risk of bacterial endocarditis Subarterial type : early repair is indicated before childhood
Partial AVSD : 1-2 years of age except CHF or 1growth failure Complete AVSD with good condition : 3-6 mo 3Complete AVSD with refractory CHF or respiratory Sx : indicated promptly Development of pulmonary vascular obstructive disease : not indicated
Critical AS in neonates : urgent (severe CHF, LV dilatation, hypertrophy) Infants and children Pressure gradient > 75mmHg Sx of angina, syncope, exercise intolerance, LVH, pressure gradient > 50mmHg Pressure gradient over 40mmHg in subvalvular lesion to prevent progression
Aortopulmonary Window
Abnormal development of aortopulmonary septum caused by incomplete formation of the right and left conotruncal ridges. Symptomatic AP window is an indication
Symptomatic infants : indicated with Dx Repair is advised before 3 mo of age (size, increased shunt, pulmonary hypertension) Old children should be operated on unless PVR render them inoperable
Ruptured sinus of Valsalva : indicated promptly because of abrupt development of CHF With VSD or VSD+AI : prompt repair is indicated Large aneurysms producing hemodynamic derangement : indicated Small and moderate sized aneurysm without symptom : not indicated
Cor Triatriatum
A rare congenital cardiac anomaly in which pulmonary veins typically enter a proximal left atrial chamber separated from the distal left atrial chamber by a diaphragm in thich there are one or more restrictive ostia (sinister, dexter) Indication for operation Restrictive aperture in the partition is an urgent indication Symptoms usually develop early, and operation is necessary in the 1st year of life Life expectancy after repair in infancy is excellent
Ebsteins anomaly
A congenital defect of tricuspid valve in which the origin of septal and posterior leaflets or both are displaced downward into the right ventricle and the leaflets are variably deformed Symptomatic Ebsteins anomaly is an indication Neonates presenting in extremes : Starnes procedure in first week Valve repair and ASD closure : with important TR moderate and severe cyanosis WPW syndromes : ablation of accessory conduction pathway
Pulmonary Stenosis
A form of RV outflow obstruction in which stenosis is usually valvar or both valvar & infundibular or only infundibular
Tetralogy of Fallot
Characterized by underdevelopment of RV infundibulum with anterior and left ward displacement Dx is an indication of operation
Symptomatic complicated in early life : Early total correction or Shunt (1-2 mo) and total correction (1 year) (1Asymptomatic uncomplicated : Total correction at 3-24 mo 3Multiple VSDs, LAD from RCA : Initial shunt and total correction
Confluent and normally distributing PAs: Complete repair in early life Palliation and repair (3-5 year) with conduit (3Confluent PAs distributing to the major segments : Pul. Segments > 15 --- need not unifocalization repair with conduit in 3-5 years 3Nonconfluent PAs distributing to the minor segment : Mmultistage operation with unifocalization Palliative shunt only
Simple TGA in neonate : arterial switch operation within 1 mo Simple TGA beyond 30 days : rapid two-stage operation twoatrial switch operation (Mustard, Senning) TGA with VSD : arterial switch operation as early TGA with VSD and LVOTO Lecompte operation at 6-18 mo 6Rastelli or Lecompte operation at 3-5 years 3-
Tricuspid Atresia
A cardiac anomaly in which RV fails to open into a ventricle through a AV valve. There is thus a univentricular AV connection PVR is an important indicator > 4 unit -- contraindicaton 2-4 unit -- BCPS < 2 unit -- Fontan operation Symptomatic in early life early shunt or PAB BCPS or hemi-Fontan at 6-12 mo hemi6Fontan at 12-24 mo 12 Nonsymptomatic Fontan candidate : 12-30 mo 12-
Dx is an indication of operation
Coexisting cardiac anomaly : not contraindication OneOne-stage repair : preferred TwoTwo-stage repair : in complicated intracardiac anomalies Single ventricle associated : alternative plan
Norwood operation
BCPS HemiHemi-Fontan
Completion Fontan
Cardiac transplantation :
Truncus Arteriosus
A cardiac anomaly in which one great artery, arising from the base of the heart by way of a truncal valve, PAs proximal to the origin of the brachiocephalic branches. Presence of truncus arteriosus : an absolute surgical indication. Neonatal repair is recommended : Homograft interposition Prosthetic valve conduit Autologous tissue reconstruction Repair should be done before 6 mo Pulmonary vascular obstructive disease : contraindication
Eisenmenger: Treatment
RULE OUT CORRECTABLE DISEASE Once diagnosis established, avoid aggressive testing as many patients die during cardiovascular procedures Diuretics prn, oxygen Definitive: Heart Lung transplant
Cardiac Transplantation
Indications
Cardiac diseases that has a poor prognosis for short-term shortsurvival(<1 year) and that is not treatable by conventional therapy
Contraindications
Severe and life-limiting coexisting medical problems lifeActive bacterial or viral infection Psychosocial family situation Presence of severely elevated PVR (>8 Wood Units) Immunologic status of the recipient panel-reactive antibody panel(PRA) > 25%
Questions?
Pediatric
exercise intolerance
Eisenmengers Syndrome
Final common pathway for all significant L R shunting in which unrestricted pulmonary blood flow leads to pulmonary vaso-occlusive disease vaso(PVOD); R L shunting/cyanosis devleops Generally need Qp:Qs >2:1
CLASSIFICATION OF CHD
Vascular Ring
Anomalies of the great arteries that compress the trachea or esophagus Double aortic arch Rt aortic arch with retroesophageal anomalous Lt. SCA and ligamentum arteriosum Rt aortic arch with retroesophageal ligamentum arteriosum Operation is indicated when : Obstructive Sx and radiologic signs of obstruction Division by thoracotomy CPB in associated cardiac anomaly
Vascular Sling
Lt. pulmonary artery arises anomalously from Rt pulmonary artery extrapericardially, form a sling around trachea Sx & Signs of obstruction : indicated Tracheal anomaly or other airway problems (+) (ASD, VSD, PDA, Lt SVC) Thoracotomy without CPB Median sternotomy with CPB
Congenital MS and MR
A developmental malformation of one or more of the components of MV apparatus, including LA wall adjacent insufficiency or a combined lesion Infancy Mild and moderate Sx without MVR : Supravalvular ring, commmissural fusion Other circumstances : only for infants with heart failure Childhood Considerations for operation are similar CHF Severe pulmonary HT MVR should be withheld whenever possible
Size of the TV : Z-value of the tricuspid valve Z< -4 --- Systemic-pulmonary artery shunt Systemic-2~-4 --- RVOT patch + shunt 2~> -2 --- RVOT patch
Two ventricle repair One and half ventricle repair Fontan procedure
Mitral Prolapse
Defined: A bulging of one or both mitral valve leaflets into the left atrium during systole T- late systolic L- apex C- midsystolic click A- ~5% normal population, asymptomatic, sudden death B- midsystolic click, most common valvular lesion, balloning/floppy valve, Marfans syndrome
A. B. C. D. E.
Aortic Regurgitation
Defined: Retrograde flow from the aorta into the left ventricle through incompetent aortic cusps T- Diastolic L- 2nd-4th left interspaces C- high-pitched, blowing high A- aortic root degeneration, rheumatic heart disease, VSD w/aortic valve prolapse (kids) B- high pulse pressure = bounding pulses
Mitral Stenosis
Defined: Obstruction of flow from left atrium to left ventricle because of a narrowed mitral orifice T- Diastolic L- Apex C- opening snap, low pitched A- Rheumatic fever B- hx of childhood rheumatic fever; presents with progressive dyspnea, pulmonary edema, hempotysis
A. B. C. D. E.
A. B. C. D. E.
Atrial septal defect Patent ductus arteriosus Pulmonic stenosis Tetralogy of Fallot Ventricular septal defect
A. B. C. D. E.
Bonus Question
The infants murmur is most likely related to which of the following: Blood flowing across the aortic valve Blood flowing from the aorta to the pulmonary artery Blood flowing from the left ventricle to the right ventricle Blood flowing from the pulmonary artery to the aorta Blood flowing from the right ventricle to the left ventricle
A. B. C. D. E.
Bonus Question
A. B. C. D. E. The infants murmur is most likely related to which of the following: Blood flowing across the aortic valve Blood flowing from the aorta to the pulmonary artery Blood flowing from the left ventricle to the right ventricle Blood flowing from the pulmonary artery to the aorta Blood flowing from the right ventricle to the left ventricle
Cause Effect LV output does not w/exercise DOE End Diastolic Volume Cardiac Dilation LV failure pulmonary venous pressure fluid transudation Pulmonary Edema, PND venous return exacerbates pulmonary vascular congestion Orthopnea
central venous pressure resistance to portal flow Hepatomegaly (nutmeg liver) RV failure venous pressure fluid transudation Ankle, sacral edema
Cardiac Tumors
I
Rheumatic Fever
Fever Erythema marginatum Valvular damage ESR Red-hot joints (polyarthritis) edSubcutaneous nodules St. Vitus Dance (chorea) Strep antibody titer (ASO )
Aschoff Bodies
Anitschkows cells
Rheumatic Fever
John Travolta career slump Makes sequel to Saturday Night Fever called Rheumatic Fever John gets
Fever Myocarditis Joint swelling Polyarthritis) (Polyarthritis) Chorea (uncontrolled dancedance-like movements of extremities
A. B. C. D. E.
A. B. C. D. E.
Pericarditis
Serous
Hemorrhagic (Invasive!)
SLE, Rhuematoid arthritis Uremia Infection (serious) (serious) MI (death of muscle fibers) fibers) Rhuematic fever Uremia
Fibrinous
May resolve without scarring May progress to chronic adhesive or constrictive pericarditis
3 syphillis- long standing syphillisDestruction of vasa vasorum Dilation of aortic root without atherosclerotic lesion = syphilitic aneurysm Calcification of ascending arch and aortic root Tree-barking- postTree-barking- postinflammatory scarring of the aorta
A. B. C. D. E.
A. B. C. D. E.
VSD
Defined: A congenital abnormality in which blood flows from high pressure LV low pressure RV through a hole T- holosystolic L- 3rd, 4th, 5th left interspaces C- harsh, loud A- alone or with other abnormalities B- infant: late cyanosis, holo/pancystolic adult: progressive pulmonary HTN, Eisengengers syndrome
Mitral Stenosis
Defined: Obstruction of flow from left atrium to left ventricle because of a narrowed mitral orifice T- Diastolic L- Apex C- opening snap, low pitched A- Rheumatic fever B- hx of childhood rheumatic fever; presents with progressive dyspnea, pulmonary edema, hempotysis
1513 Leonardo da Vinci describes perforating channel in atrial septum 1875 Rokitansky first describes ASD 1941 Bedford et al describe clinical features 1950s first successful open surgical repair 1980s1980s- present - transcatheter approaches to repair
ASD - Epidemiology
Embryologic Development
Braunwauld 6th ed
ASD - Anatomy
Ostium Secundum -75% Ostium Primum - 15% Sinus Venosus - 10%
Braunwauld 6th ed
Ostium Secundum
MVP (10-20%) (10IRBBB, RAD MR/ cleft AMVL LAD, 1st degree AVB 75%
Ostium Primum
Sinus Venosus
anomalous pulm venous drainage into RA or vena cavae junctional/low atrial rhythm
Physiologic Consequences
Shunt Flow
L R shunting results in diastolic overload of RV and RV increased pulmonary blood flow RV dilatation/failure and rarely severe pulm HTN (Eisenmengers) may ensue over time ~5% With age, deterioration chiefly due to 1
decrease LV compliance, increased L R shunt increase in atrial arrhythmias pulm HTN develops, RV volume + pressure OL
NEJM 1995
1Perloff,
Clinical Symptoms
Often asymptomatic until 3-4th decade for 3moderatemoderate-large ASD, may present later in life for initially smaller ASD Fatigue DOE Atrial arrhythmias Paradoxical Embolus Recurrent Pulmonary infections
Physical Signs
S2 wide/fixed splitting RV/PA palpable impulse (if lg defect) systolic ejection murmur 2nd L ICS midmid-diastolic TV rumble
ECG
ECHO
Subcostal view of Intraatrial Septum Color Flow/ Contrast Good for secundum, primum
Catheterization
Catheterization/Oximetry
Treatment
Mortality 1-3% in most series 1 PVR > 6-8 Woods Units - Contraindication 6
Interventional
Only for secundum defects 94-96% success (Amplatzer) 94
2727-32 year followup divided into groups according to age (<11, 121224, 25-40, and >41)and presence of mod-sev 25modpulm HTN (PA s>40) at time of cath excluded primum ASD 75% symptomatic, older pt more likely to be on med Rx (Dig, diuretic, Quinidine)
Survival Curves
28 deaths
13 (48%) Cardiac death 5 (19%) CVA (all in afib) 6 (21%) Noncardiac (cancer, sepsis, resp fail)
Data on PVR available on only 42% of pt and was not included in statistical analysis A stated purpose of study was to determine employability and insurability of these pt and was not meant to be a guideline Led to consensus that repair <age 24 had nl mortality, between age 25-41 good survival but less than expected, and > age 41 had 25substantial increase in mortality Pts advised to have ASD repair because untreated prognosis thought to be poor
Surgical (48)
Presentation Follow up
CV Death NYHA I NYHA II NYHA III Atrial Fibrillation 25 (74%) 9 (26%) 0 (0%) 7 (20%)
Earlier data showing high morbidity and reduced survival was based on a group of highly selected pt b/c florid clinical signs of ASD were needed before catheterization considered (pre ECHO) In asymptomatic patients, ASD repair offered no benefit with regard to mortality, morbidity or progression to atrial arrhythmia Limitations: uncontrolled study, advanced pulm HTN excluded (these pt do better with surgery), 22% of original pt lost to followup
Children with sxs ASD repair Asymptomatic close followup and repair when sxs/hemodynamic deterioration Older pt >25, surgery may not benefit in terms of sxs/pulm HTN/mortality Questioned benefit of routine surgical repair of older pt with ASD
Sought to address issue of benefit/lack of benefit to ASD repair in middle aged-elderly pt agedRetrospective, 179 pt with ASD dx > age 40 between 19661966-1991 47% surgery 53 % medical Mean followup of 8.9+-5.2 years 8.9+Women 70%
Results
Medical
10yr Surv. NYHA worse NYHA better1 Afib/flutter
169%
Surgery
95% p=.02 11% 32% 15%
Konstantinides, et al - Summary
31% reduction in mortality among symptomatic pt , age > 40 with surgical repair Symptomatic improvement in NYHA functional class and less deterioration among surgically treated pt No effect on atrial arrhythmias First study to show benefit of surgery in older pt with ASD/ sxs Limitations retrospective, nonrandomized; excluded pt with CAD or severe MR (prev study by same author showed no benefit in unselected pt1)
et al. Circulation 1994
1Konstantinides,
Conclusions
Age < 25, sxs, significant ASD Repair Older age not contraindication and evidence supports mortality, symptomatic benefit for ASD repair in symptomatic pt with significant ASD
1513 Leonardo da Vinci describes perforating channel in atrial septum 1875 Rokitansky first describes ASD 1941 Bedford et al describe clinical features 1950s first successful open surgical repair 1980s1980s- present - transcatheter approaches to repair
ASD - Epidemiology
ASD - Anatomy
Ostium Secundum -75% Ostium Primum - 15% Sinus Venosus - 10%
Braunwauld 6th ed
Ostium Secundum
MVP (10-20%) (10IRBBB, RAD MR/ cleft AMVL LAD, 1st degree AVB 75%
Ostium Primum
Sinus Venosus
anomalous pulm venous drainage into RA or vena cavae junctional/low atrial rhythm
Physiologic Consequences
Shunt Flow
L R shunting results in diastolic overload of RV and RV increased pulmonary blood flow RV dilatation/failure and rarely severe pulm HTN (Eisenmengers) may ensue over time ~5% With age, deterioration chiefly due to 1
decrease LV compliance, increased L R shunt increase in atrial arrhythmias pulm HTN develops, RV volume + pressure OL
NEJM 1995
1Perloff,
Clinical Symptoms
Often asymptomatic until 3-4th decade for 3moderatemoderate-large ASD, may present later in life for initially smaller ASD Fatigue DOE Atrial arrhythmias Paradoxical Embolus Recurrent Pulmonary infections
Physical Signs
S2 wide/fixed splitting RV/PA palpable impulse (if lg defect) systolic ejection murmur 2nd L ICS midmid-diastolic TV rumble
ECG
ECHO
Subcostal view of Intraatrial Septum Color Flow/ Contrast Good for secundum, primum
most common ACHD (6-10%) (6RAD associated with other endocardial cushion defects (cleft AV valves, inlet type VSD) LAD large, associated with anomalous pulmonary venous drainage (usually R superior PV) associated with unroofed coronary sinus
Primum
Sinus Venosus
ASDASD- Anatomy/Prevalence
Secundum 75% Primum 15% Sinus Venosus 10% Cor Sinus (rare)
ASD - Clinical
Symptomatic
Auscultation in ASD
Increased flow across the pulmonary valve produces a systolic ejection murmur and fixed splitting of the second heart sound. Fixed splitting of S2 may in part be due to delayed right bundle conduction. Increased flow across the TV produces a diastolic rumble at the mid to lower right sternal border.
Older pt loses pulm ejection murmur as shunt becomes bidirectional signs of pulm HTN/ CHF may predominate
ASD: Therapy
Percutaneous Closure
only for secundum (contra in others) adequate superior/inferior rim around ASD no R-L shunting RGood prognosis:
closure age < 25, PA pressure <40 If >25 or PA>40, decreased survival due to CHF, stroke, and afib
Surgical Closure
CLASSIFICATION OF CHD
L R SHUNTS
Defects connecting arterial & venous circulation SVR > PVR PBF pulmonary blood flow pulmonary congestion CHF susceptibility to RTI Long standing L-R shunts LPHT PVR > SVR RR-L shunt Eisenmengers syndrome
Catheterization
Catheterization/Oximetry
Treatment
Mortality 1-3% in most series 1 PVR > 6-8 Woods Units - Contraindication 6
Interventional
Only for secundum defects 94-96% success (Amplatzer) 94
untreated mortality
noted that pattern of progressive disability began around 3rd decade and included dyspnea, cardiac failure, atrial fibrillation and pulmonary HTN 67 pt 1943-1963, all survived to age 40 194340% died/disabled by 5th decade 90% older than 60 2,3 were severely disabled
1965 Markman4
et al. Br Heart J 1941; Campbell M, et al. Br Heart J 1957,1970 4 Markman P, et al. Q J Med 1965
1Bedford,
2727-32 year followup divided into groups according to age (<11, 121224, 25-40, and >41)and presence of mod-sev 25modpulm HTN (PA s>40) at time of cath excluded primum ASD 75% symptomatic, older pt more likely to be on med Rx (Dig, diuretic, Quinidine)
Survival Curves
28 deaths
13 (48%) Cardiac death 5 (19%) CVA (all in afib) 6 (21%) Noncardiac (cancer, sepsis, resp fail)
Data on PVR available on only 42% of pt and was not included in statistical analysis A stated purpose of study was to determine employability and insurability of these pt and was not meant to be a guideline Led to consensus that repair <age 24 had nl mortality, between age 25-41 good survival but less than expected, and > age 41 had 25substantial increase in mortality Pts advised to have ASD repair because untreated prognosis thought to be poor
Surgical (48)
Presentation Follow up
CV Death NYHA I NYHA II NYHA III Atrial Fibrillation 25 (74%) 9 (26%) 0 (0%) 7 (20%)
Earlier data showing high morbidity and reduced survival was based on a group of highly selected pt b/c florid clinical signs of ASD were needed before catheterization considered (pre ECHO) In asymptomatic patients, ASD repair offered no benefit with regard to mortality, morbidity or progression to atrial arrhythmia Limitations: uncontrolled study, advanced pulm HTN excluded (these pt do better with surgery), 22% of original pt lost to followup
Children with sxs ASD repair Asymptomatic close followup and repair when sxs/hemodynamic deterioration Older pt >25, surgery may not benefit in terms of sxs/pulm HTN/mortality Questioned benefit of routine surgical repair of older pt with ASD
Sought to address issue of benefit/lack of benefit to ASD repair in middle aged-elderly pt agedRetrospective, 179 pt with ASD dx > age 40 between 19661966-1991 47% surgery 53 % medical Mean followup of 8.9+-5.2 years 8.9+Women 70%
Results
Medical
10yr Surv. NYHA worse NYHA better1 Afib/flutter
169%
Surgery
95% p=.02 11% 32% 15%
Konstantinides, et al - Summary
31% reduction in mortality among symptomatic pt , age > 40 with surgical repair Symptomatic improvement in NYHA functional class and less deterioration among surgically treated pt No effect on atrial arrhythmias First study to show benefit of surgery in older pt with ASD/ sxs Limitations retrospective, nonrandomized; excluded pt with CAD or severe MR (prev study by same author showed no benefit in unselected pt1)
et al. Circulation 1994
1Konstantinides,
Conclusions
Age < 25, sxs, significant ASD Repair Older age not contraindication and evidence supports mortality, symptomatic benefit for ASD repair in symptomatic pt with significant ASD
--23-year-old woman with tricuspid atresia after insertion of Glenn and Fontan shunts who presented with new atrial fibrillation
--17-year-old boy with transposition of great arteries after Mustard procedure and history of pulmonary vein repair who presented with new onset of hemoptysis
--23-year-old woman with tricuspid atresia after insertion of Glenn and Fontan shunts who presented with new atrial fibrillation
1. Drugs
Alcohol Amphetamines Anticonvulsants Chemotherapy Sex hormone Thalidomide Retinoic acid
4. Others
Factors
Malformation
Trisomy 21
Advanced age
Maternal CHD Various Diabetes mellitus VSD, TGA, cardiomyopathy SLE Heart block Phenylketonuria TOF, VSD, COA, HLHS Viruses Teratogenic, myocarditis (*cytomegalovirus, herpes, coxsacki B, parvovirus)
Drug
Malformation
Diphenylhydantoin PS, AS Trimetadione VSD, TOF, TGA, HLHS Thalidomode TOF, Truncus arteriosus Lithium Ebstein anomalies Alcohol VSD, ASD, PDA, TOF Amphetamine VSD, ASD, PDA, TGA Birth control pills VSD, TOF, TGA
2. Development of heart
1) Position and cardiac tube at 23 days Heart beating at 26 days. 2) Formation of heart loop at 8 somites 3) Formation of ventricle 4) Development of sinus venosus
3. 4. 5. 6.
Formation of cardiac septa Formation of cardiac valves Formation of arterial system Formation of great systemic veins
Lt to Rt Shunt
PDA ASD VSD AVSD Abn. PV return
( 53 % )
17 % 16.5 % 13 % 3.5 % 3 %
4. Obstructive Lesion
( 15 % )
2. Rt to Lt Shunt
TOF TA PA+VSD PA+IVS TGA Univ. Ht. Atrial isomerism DORV Truncus Corrected TGA
(11 % )
4.5 % 3 % 2.5 % 0.5 %
3. Admixture Lesion
5 % 5 % <2 % <2 % 0.8 % < 0.5 %
( 15 % )
Coarctation 9.5 % PS 2 % MS etc. 1.5 % LVOTO 1.3 % HLHS 0.9 % IAA 0.6 % 5. Valvular Lesion Ebstein <1 % AR < 0.5 % MR < 0.5 % SV aneurysm < 0.5 % 6. Miscellaneous Arrhythmia 5 % Vascular ring 0.5 %
2. Children
1) Murmur 2) Symptoms of CHF exercise intolerance, dyspnea on exertion, frequent URI, palpitation 3) Syncope, chest pain 4) Symptoms of Hypoxemia cyanosis, hypoxic spell,clubbing
To Be Corrected in Neonate
Critical AS Hypoplastic left heart syndrome Mitral valve hypoplasia Aortic valve and arch hypoplasia Hypoplastic left ventricle Interrupted aortic arch Symptomatic COA TGA Obstructive TAPVC Truncus Arteriosus PA with IVS PA with VSD Other symptomatic complex heart diseases
To Be Corrected in Infancy(I)
Cardiac anomalies with pulmonary outflow tract obstruction
Double inlet ventricle Critical PS Tricuspid atresia DORV DOLV TGA TOF PA with or without VSD Corrected TGA
To Be Corrected in Infancy(II)
Cardiac anomalies with CHF
Large VSD AVSD Double inlet ventricle Tricuspid atresia TAPVC COA DOLV PA with IVS Corrected TGA Cardiac mass
TGA Truncus arteriosus Severe AS, AR Severe MS, MR Interrupted aortic arch DORV Aortopulmonary window ALCAPA PDA TAPVR
ASD AS(LVOTO) Partial AVSD Ebsteins anomaly VSD with PS Coronary artery anomaly (A-V fistula) (AAnomalous pulmonary venous drainage Valvular heart diseases Complex anomalies with previous palliation Late presenting cardiac anomalies Cardiomyopathy
Dx is an indication of operation
Septation : 1-2 years 1enlarged dominant ventricle two competent nonstenotic AV valve no pulmonary or systemic outflow obstruction Fontan operation : 1-2 years 1< 3-5 mo 3--- systemic outflow obstruction 6 - 12 mo --- BCPS 1 2 years --- Fontan operation Cardiac transplantation
Diagnosis is not an indication for operation Coexisting cardiac anomalies are indications Large VSD always present Pulmonary stenosis is usual Subaortic stenosis may occur Tricuspid atresia or TV hypoplasia in half
Atrial Isomerism
A condition which the right sided and left sided atria, normally morphologically different, are morphologically similar. Surgical treatment is indicated with coexisting anomaly, not by the atrial isomerism. Intraventricular complex repair Complex atrial baffling Fontan type repair Palliative operation
--23-year-old woman with Down syndrome and history of atrial ventricular canal who presented with new onset of chest pain
--23-year-old woman with Down syndrome and history of atrial ventricular canal who presented with new onset of chest pain