Vous êtes sur la page 1sur 298

CONGENITAL HEART DISEASE

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

Relative Frequency of Lesions


Ventricular septal defect Atrial septal defect (secundum) Patent ductus arteriosus Coarctation of aorta Tetralogy of Fallot Pulmonary valve stenosis Aortic valve stenosis Transposition of great arteries Hypoplastic left ventricle Hypoplastic right ventricle Truncus arteriosus Total anomalous pulm venous return Tricuspid atresia Double-outlet right ventricle Others 25-30 6-8 6-8 5-7 5-7 5-7 4-7 3-5 1-3 1-3 1-2 1-2 1-2 1-2 5-10

CHD CLASIFICARE
   

-SHUNT L-R L-SHUNT R-L R-NON SHUNT -COMPLEX

CLASSIFICATION OF CHD

CLASSIFICATION OF CHD


L - R SHUNTS INCLUDE :
ASD  VSD  PDA



7.5% of CHD COMMONEST CHD 25% 7.5% of CHD

Common in premature infants Often seen with trisomy 21

ENDOCARDIAL CUSHION DEFECT - 3%




AORTOPULMONARY WINDOW

Noncyanotic CHD (L pR)


Atrial septal defects (ASD) Ventricular septal defects (VSD) Patent ductus arteriosus (PDA) Obstruction to blood flow
Pulmonic stenosis (PS) Aortic stenosis (AS) Aortic coarctation

ATRIAL SEPTAL DEFECT

ASD - Clinical


Majority repaired in childhood, but may present in adolescence/adulthood Asymptomatic




murmur, abnl ECG/CXR dyspnea/CHF CVA/emboli Atrial Fibrillation

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

Atrial Septal Defect




  

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

VENTRICULAR SEPTAL DEFECT

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

VENTRICULAR SEPTAL DEFECT

VSD

Ventricular Septal Defect (VSD)


Small VSDs, the chest radiograph is usually normal

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.

ATRIOVENTRICULAR CANAL DEFECTS

ATRIOVENTRICULAR CANAL DEFECT

PATENT DUCTUS ARTERIOSUS

Patent Ductus Arteriosus


Defined: Failure of the duct between pulmonary artery and aorta to close  T- Continuous  L- upper left sternal border  C- machine-like machine A- left right shunt, cyanosis


B- infants, machine-like, continuous machine-

murmur

Patent Ductus Arteriosus


 

Small defect no symptoms. Large defect:


    

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

Coarctation of Aorta: Clinical




Most repaired, but adult presentation may be:


 

HTN murmur (continuous or systolic murmur heard in back or SEM/ejection click of bicuspid AV)




weak/delayed LE pulses Rib notching on CXR pathognomonic

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
  

Aortic Aneurysm/dissection CHF Premature CADz

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

Edmunds Cardiac Surgery in the Adult, Ch 47

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


Variability correlates with degree of RVOT obstruction and size/anatomy of PA

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


Tetralogy: Surgical Treatment




Systemic Pulmonary Shunt


  

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


SCD 25-100 fold 25   

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 OF GREAT ARTERY

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

 

systemic (tricuspid) atrioventricular valve regurgitation




systemic (RV) ventricular failure


 

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

L-type transposition: complications




 

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

Severe AV (tricuspid) regurgitation TVR


 

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

Ebsteins: Clinical Presentation




Pediatric


murmur atrial arrhythmias murmur cyanosis




Adult (unrepaired with ASD)


  

R L shunt NOT due to PulmHTN but TR jet directed across ASD

exercise intolerance

Surgery in pts with significant TR/sxs

Massive cardiomegaly, mainly due to RAE

PULMONARY HYPERTENSION


EISENMENGER SYNDROM

Eisenmenger Syndrom- Pulmonary SyndromHypertension


 

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


Sxs +polycythemia phlebotomy




Careful if microcytosis, strongest predictor of cerebrovascular events

   

RULE OUT CORRECTABLE DISEASE Once diagnosis established, avoid aggressive testing as many patients die during cardiovascular procedures Diuretics prn, oxygen Definitive: Heart Lung transplant


Prostacyclin therapy may delay, expensive

Eisenmenger Complications
   

Coagulopathy/platelet consumption Brain abcesses Cerebral microemboli Airway hemorrhage




especially moving from lower higher altitudes (air travel, mountains)

COARTATION af the AORTA

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


PULMONARY VALVE STENOSIS

Surgery for Congenital Heart Diseases

Surgical Indications and Optimal Timing of Operation

Palliative Surgery


Systemic pulmonary artery shunt


BlalockBlalock-Taussig shunt Unifocalization and shunt Cavopulmonary shunt (BCPS)

RVOT reconstruction
Valvotomy Patch widening Valved conduit

 

Pulmonary artery banding Atrial septectomy

Systemic SystemicPulmonary Artery Shunt


Systemic Systemicpulmonary artery shunt is indicated due to age, size, anatomy or other conditions when:


 

Complex anomaly with severe cyanosis, irritability, hypoxic episode Critically ill neonates or infants due to decreased pulmonary f Facilitating growth of hypoplastic pulmonary artery

Pulmonary Artery Banding


Pulmonary artery banding is indicated to decrease pulmonary blood flow & protect vascular disease Control of congestive heart failure Complex or multiple VSD (+/- coarctation) (+/Single ventricle, Tricuspid atresia without PS CPB medically contraindicated Protection of pulmonary vascular bed Single ventricle Fontan operation Preparation of LV for arterial switch operation TGA with IVS / restrictive VSD when:

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
 

Anatomic correction Physiologic correction

NonNon-open Heart Surgery


Palliative procedure Corrective procedure PDA COA Vascular ring and sling Coronary artery anomalies Stenotic valvular diseases
 

    

Inflow occlusion technique Instrumental dilatation

Patent Ductus Arteriosus


Open communication usually between upper descending Ao and proximal portion of LPA
   

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

Coarctation of the Aorta


Congenital narrowing of upper thoracic aorta adjacent to the ductus arteriosus Operation is indicated when : Reduction of luminal diameter > 50% Upper body HT > 150mmHg in young infant With CHF at any age COA with VSD Two stage repair One stage repair COA with other important intracardiac defects One stage repair

Open Heart Surgery

Total Anomalous Pulmonary Venous Connection


These are no direct connection between any pulmonary vein and the LA. But rather, all the pulmonary veins connect to the RA or one of its tributaries Dx is an indication of operation  Immediate repair with Dx in any ill neonate : Preop preparation is not needed  Repair should be done nearly always before 6 mo  Dx at 6-12 mo : prompt repair is indicated 6-

Ventricular Septal Defect


A hole (or multiple holes) between Lt & Rt ventricle
   

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

Atrioventricular Septal Defect


Abnormalities of atrioventricular valve form & function and interatrial & interventricular communication from maldevelopment of the endocardial cushions Presence of AVSD : indicated with Dx

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

Congenital Aortic Stenosis


The various forms of LVOTO occur in combination
with other cardiac lesions (IAA, COA, MV anomalies, LV hypoplasia) and obstructive types are supravalvular, valvular, subvalvular, intraventricular


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

Aneurysm of Sinus of Valsalva


Thin walled, tubular outpouchings, nearly always Rt sinus or adjacent half of the noncoronary sinus and with an entirely intracardiac course, that may rupture into the right (rarely left) heart chamber to form a fistula
   

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

Critical PS in neonate : indicated with Dx


Percutaneous balloon valvotomy Valvotomy with CPB Transannular RVOT patch widening Valvotomy with inflow occlusion technique PS in infants and children : indicated with Sx & Pr gradient over 50mmHg Surgical treatment is not indicated with mild stenosis

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

Pulmonary Atresia with VSD


These are no luminal continuity between RV & pulmonary trunk among the group of TOF. It is usually congenital, but may be acquired. Dx is an indication of operation

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

Transposition of Great Arteries


A cardiac anomaly in which the Ao arises entirely or in large part from the RV, and PA from LV (atrioventricular concordant connection and ventriculoarterial discordant connection)


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-

Interrupted Aortic Arch


Complete luminal and anatomic discontinuity between two segments of the aortic arch, and generalized narrowing of LVOT, posterior malalignment, muscle of Moulaert, small aortic annulus, aortic hypoplasia

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

Hypoplastic Left Heart Syndrome


A wide spectrum of cardiac anomaly with various degree of hypoplasia of the structure of the left side of the heart Dx is an indication of operation

First stage palliation : 1-30days 1-

Norwood operation

Second stage palliation : 6-12 mo 6-

BCPS HemiHemi-Fontan

Third stage correction : 18-24 mo 18-

Completion Fontan

Cardiac transplantation :

Aortic diameter < 2.5 mm

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

 

Congenitally Corrected TGA


A cardiac anomaly with ventriculoarterial discordant connection & atrioventricular discordant connection. The circulatory pathways are therefore in series The presence of CCTGA per se is not an indication. With VSD : indications for VSD With VSD + PS : indications for TOF Isolated TR : indication for MR With complete heart block : pacing Fontan procedure indicated : straddling tricuspid valve complete AVSD left sided tricuspid valve incompetence Double Switch operation : anatomic correction

    

Coronary Arteriovenous Fistula


Direct communication between coronary artery and lumen of any one of cardiac chamber, or coronary sinus, or its tributary veins, or SVC, PAs, or Pulmonary veins close to the heart Indicated unless the shunt is small (Qp/Qs<1.3) Increase in size Sx and heart failure SBE Low probability of spontaneous closure Safety and efficacy of operation Techniques Suture ligation without CPB Closure of transcoronary artery with CPB Transcardiac chamber closure with CPB

Eisenmenger: Treatment


Sxs +polycythemia phlebotomy




Careful if microcytosis, strongest predictor of cerebrovascular events

   

RULE OUT CORRECTABLE DISEASE Once diagnosis established, avoid aggressive testing as many patients die during cardiovascular procedures Diuretics prn, oxygen Definitive: Heart Lung transplant


Prostacyclin therapy may delay, expensive

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?

Congestive Cardiac Failure

 

Enlarged heart Plethoric lung fields specially at bases

Ebsteins: Clinical Presentation




Pediatric


murmur atrial arrhythmias murmur cyanosis




Adult (unrepaired with ASD)


  

R L shunt NOT due to PulmHTN but TR jet directed across ASD

exercise intolerance

Surgery in pts with significant TR/sxs

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

Patent Ductus Arteriosus


Defined: Failure of the duct between pulmonary artery and aorta to close  T- Continuous  L- upper left sternal border  C- machine-like machine A- left right shunt, cyanosis  B- infants, machine-like, continuous murmur machine-

Patent Ductus Arteriosus


Defined: Failure of the duct between pulmonary artery and aorta to close  T- Continuous  L- upper left sternal border  C- machine-like machine A- left right shunt, cyanosis  B- infants, machine-like, continuous murmur machine-

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

ASD and PAPVR


A hole of variable size in the atrial septum and is most common cardiac malformation with various location of defect, fossa ovalis, posterior, ostium, primum, coronary sinus, subcaval (sinus venosus) Uncomplicated ASD or of PAPVC with RV volume overload (Qp/Qs>1.5 or 2.0) : an indication  Scimitar syndrome  Isolated PAPVC  Optimal age : under 5 years but recently 1-2 years 1to avoid RV volume overload

Unroofed Coronary Sinus Syndrome


A spectrum of cardiac anomalies in which part or all of the common wall between the coronary sinus and left atrium is absent Operation is advisable with diagnosis when  With persistent Lt SVC Arterial desaturation Risk of cerebral embolism Good results of operation  Without persistent Lt SVC(Coronary sinus ASD) Same as for other type ASD  Associated with other major cardiac anomalies Clear indication for operation

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

PA with Intact Ventricular septum


A cardiac anomaly in which the pulmonary valve is atretic, coexisting with variable degree of right ventricle and tricuspid valve hypoplasia Dx is an indication of operation

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

Evaluation after 6-12 mo after initial procedure : 6-

Two ventricle repair One and half ventricle repair Fontan procedure

Double Outlet Right Ventricle


A congenital cardiac anomalies which both great arteries rise wholly or in large part from the RV. It is, then, a type of ventriculoarterial connection. Dx is an indication of operation Simple DORV with subaortic VSD : repair by 6 mo with PS --- repair like TOF DORV with subpulmonic VSD (Taussig-Bing heart) : (Taussigarterial switch operation within 1 mo with PS --- REV or Rastelli operation at 3-5 years 3 DORV with noncommitted VSD : VSD enlargement and intraventricular tunnel Fontan operation

Possible Exam Question


A term baby is sent home with his mother after delivery. The pregnancy was normal. No anomalies were noted at the later, time of birth. Five weeks later, the mother brings the baby birth. to the clinic because the baby has difficulty breathing and occasionally turns blue. You hear a pansystolic murmur blue. which is probably due to a(an): A. Hypertrophic subaortic stenosis B. Hypoplastic left heart syndrome C. Coarctation of the aorta D. Ventricular septal defect E. Bicuspid aortic valve

Possible Exam Question


A term baby is sent home with his mother after delivery. The pregnancy was normal. No anomalies were noted at the time of birth. Five weeks later, the mother brings the baby to the clinic because the baby has difficulty breathing and occasionally turns blue. You hear a pansystolic murmur which is probably due to a(an): A. Hypertrophic subaortic stenosis B. Hypoplastic left heart syndrome C. Coarctation of the aorta D. Ventricular septal defect E. Bicuspid aortic valve

Possible Exam Question


A 58-year-old man develops deep venous thrombosis during a 58-yearhospitalization for prostatectomy. He exhibits decreased mental status with right hemiplegia, and a CT scan of the head suggests an acute cerebral infarction in the distribution of the left middle cerebral artery. A chest radiograph reveals cardiac enlargement and prominence of the main pulmonary arteries that suggests pulmonary hypertension. His hypertension. serum troponin I is <0.4 ng/mL. Which of the following lesions is most likely to be present on echocardiography: A. Coarctation of the aorta B. Tetralogy of Fallot C. Ventricular septal defect D. Pulmonic stenosis E. Dextrocardia

Possible Exam Question


A 58-year-old man develops deep venous thrombosis during a hospitalization for prostatectomy. He exhibits decreased mental status with right hemiplegia, and a CT scan of the head suggests an acute cerebral infarction in the distribution of the left middle cerebral artery. A chest radiograph reveals cardiac enlargement and prominence of the main pulmonary arteries that suggests pulmonary hypertension. His serum troponin I is <0.4 ng/mL. Which of the following lesions is most likely to be present on echocardiography: A. Coarctation of the aorta B. Tetralogy of Fallot C. Ventricular septal defect D. Pulmonic stenosis E. Dextrocardia

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

Possible Exam Question


A 19-year-old woman is found to have a cardiac murmur 19-yearcharacterized by a mid systolic click. An echocardiogram click. demonstrates mitral insufficiency with upward displacement of one leaflet. There is aortic root dilation to 4 cm. She has a dislocated right ocular crystalline lens. She dies suddenly and unexpectedly. The medical examiner finds a prolapsed mitral valve with elongation, thinning, and rupture of chordae tendineae. A mutation involving tendineae. which of the following genes is most likely to be present in this patient: BetaBeta-myosin CFTR FGFR Fibrillin Spectrin

A. B. C. D. E.

Possible Exam Question


A 19-year-old woman is found to have a cardiac murmur characterized by a mid systolic click. An echocardiogram demonstrates mitral insufficiency with upward displacement of one leaflet. There is aortic root dilation to 4 cm. She has a dislocated right ocular crystalline lens. She dies suddenly and unexpectedly. The medical examiner finds a prolapsed mitral valve with elongation, thinning, and rupture of chordae tendineae. A mutation involving which of the following genes is most likely to be present in this patient: A. Beta-myosin B. CFTR C. FGFR D. Fibrillin E. Spectrin

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

Possible Exam Question


A 67-year-old man presents to his doctors office for an 67-yearinsurance physical. During blood pressure measurement, the nurse notes that systolic sounds are heard with the cuff completely deflated. The blood pressure is 180/60 mm Hg. highPhysical examination reveals bounding pulses and a high-pitched, blowing diastolic murmur, heard best along the left sternal murmur, border. border. Which of the following are the most likely diagnosis? A. Aortic regurgitation B. Aortic valve obstruction C. Cardiac Tamponade D. Heart failure E. Hypovolemia

Possible Exam Question


A 67-year-old man presents to his doctors office for an insurance physical. During blood pressure measurement, the nurse notes that systolic sounds are heard with the cuff completely deflated. The blood pressure is 180/60 mm Hg. Physical examination reveals bounding pulses and a highpitched, blowing diastolic murmur, heard best along the left sternal border. Which of the following are the most likely diagnosis? A. Aortic regurgitation B. Aortic valve obstruction C. Cardiac Tamponade D. Heart failure E. Hypovolemia

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

Possible Exam Question


A 30 year-old Hispanic woman reports to her primary care physician yeardyspnea. twocomplaining of progressive dyspnea. She reports she has a two-year history of exertional shortness of breath that has now worsened and affects her even with modest amounts of activity. Occasionally, a dry cough accompanies her dyspnea. Today, she had a mild episode of hemoptysis, which prompted her hemoptysis, visit. She denies fever, chills, or sputum production. Her medical history is significant for a febrile illness with sore throat and joint pain at the age of 15 in Mexico. On examination, she is a well-developed female in no acute Mexico. welldistress. A crescendo diastolic rumble is present at the apex of the heart and bases. fine crackles are auscultated at the lung bases. Which of the following is the most likely diagnosis? Mitral stenosis Myocardial infarcion Pneumonia Pulmonary effusion Pulmonary embolus

A. B. C. D. E.

Possible Exam Question


A 30 year-old Hispanic woman reports to her primary care physician complaining of progressive dyspnea. She reports she has a two-year history of exertional shortness of breath that has now worsened and affects her even with modest amounts of activity. Occasionally, a dry cough accompanies her dyspnea. Today, she had a mild episode of hemoptysis, which prompted her visit. She denies fever, chills, or sputum production. Her medical history is significant for a febrile illness with sore throat and joint pain at the age of 15 in Mexico. On examination, she is a well-developed female in no acute distress. A crescendo diastolic rumble is present at the apex of the heart and fine crackles are auscultated at the lung bases. Which of the following is the most likely diagnosis? Mitral stenosis Myocardial infarcion Pneumonia Pulmonary effusion Pulmonary embolus

A. B. C. D. E.

Patent Ductus Arteriosus


Defined: Failure of the duct between pulmonary artery and aorta to close  T- Continuous  L- upper left sternal border  C- machine-like machine A- left right shunt, cyanosis  B- infants, machine-like, continuous murmur machine-

Possible Exam Question


An x-ray performed on a newborn infant shows enlargement xof the left ventricle and left atrium as well as dilation of the aorta. Echocardiographic studies demonstrate volumevolumeoverloading of the left ventricle. Cardiac auscultation reveals the presence of a continuous, machine-like murmur. Which of machinethe following is the most likely diagnosis?
A. B. C. D. E.

Atrial septal defect Patent ductus arteriosus Pulmonic stenosis Tetralogy of Fallot Ventricular septal defect

Possible Exam Question


An x-ray performed on a newborn infant shows enlargement of the left ventricle and left atrium as well as dilation of the aorta. Echocardiographic studies demonstrate volume-overloading of the left ventricle. Cardiac auscultation reveals the presence of a continuous murmur. Which of the following is the most likely diagnosis? 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

Congestive Heart Failure


  

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

Congestive Heart Failure




central venous pressure resistance to portal flow Hepatomegaly (nutmeg liver) RV failure venous pressure fluid transudation Ankle, sacral edema

Cardiac Tumors


Most common tumor = Metastasis

I
  

Most common 1 adult tumor = Myxoma 1




BallBall-valve obstruction in Left Atrium (LA

Dodgers) Most common 1 pediatric tumor = 1 rhabdomyoma




Associated with tuberous sclerosis

Rheumatic Fever
       

Fever Erythema marginatum Valvular damage ESR Red-hot joints (polyarthritis) edSubcutaneous nodules St. Vitus Dance (chorea) Strep antibody titer (ASO )

Aschoff Bodies


Granulomas with giant cells Activated histiocytes

Anitschkows cells


Two RHussians with RHumatic heart disease

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

St. Vitus Dance

Possible Exam Question


A 42-year-old woman has increasing congestive heart 42-yearfailure. As a child she suffered recurrent bouts of pharyngitis with group A beta hemolytic streptococcal infections. The cardiac valves most likely to be affected are: Aortic and tricuspid Mitral and pulmonic Aortic and pulmonic Tricuspid and pulmonic Mitral and aortic

A. B. C. D. E.

Possible Exam Question


A 42-year-old woman has increasing congestive heart failure. As a child she suffered recurrent bouts of pharyngitis with group A beta hemolytic streptococcal infections. The cardiac valves most likely to be affected are: Aortic and tricuspid Mitral and pulmonic Aortic and pulmonic Tricuspid and pulmonic Mitral and aortic

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

TB (think hemoptysis) hemoptysis) Malignancy (aggressive)

Fibrinous


 

May resolve without scarring May progress to chronic adhesive or constrictive pericarditis

Syphilitic Heart Disease


  

 

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

Possible Exam Question


Examination of an autopsy specimen from a Mexican immigrant demonstrates a heart with massive dilation of the aortic root and adjacent aortic arch. Opening the aorta reveals a distinctive wrinkling of the intimal surface. surface. If a histological section through the aortic wall is made, which of the following will be seen? A heavy eosinophilic infiltrate Fibrinoid necrosis with a neutrophilic infiltration Focal fragmentation of elastic elements Obliterative endarteritis of vasa vasorum RingRing-like calcification of the vessel media

A. B. C. D. E.

Possible Exam Question


Examination of an autopsy specimen from a Mexican immigrant demonstrates a heart with massive dilation of the aortic root and adjacent aortic arch. Opening the aorta reveals a distinctive wrinkling of the intimal surface. If a histological section through the aortic wall is made, which of the following will be seen? A heavy eosinophilic infiltrate Fibrinoid necrosis with a neutrophilic infiltration Focal fragmentation of elastic elements Obliterative endarteritis of vasa vasorum Ring-like calcification of the vessel media

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

Historical Perspectives - 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
 

1/3 of all Adult congenital heart disease 2-3:1 female to male

Embryologic Development

Braunwauld 6th ed

ASD - Anatomy
Ostium Secundum -75% Ostium Primum - 15% Sinus Venosus - 10%

Braunwauld 6th ed

Associated conditions/ECG abnormalities




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
  

Size of defect Relative compliance of ventricles Relative resistance of pulmonary/systemic circulation

  

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
 

Oximetry Shunt Ratio (Qp/Qs)

Grossman, Cardiac Cath. 6th ed Ch 9

Catheterization/Oximetry

Grossman; Keane JF et al, Grossman Cardiac Cath.6th ed Chs 9,34

Treatment
 

Medical : diuretics, ACEI, Aldactone Repair


Consider when sxs, Qp:Qs>1.5  Surgical


Mortality 1-3% in most series 1 PVR > 6-8 Woods Units - Contraindication 6

Interventional
Only for secundum defects  94-96% success (Amplatzer) 94

Percutaneous Devices used for Closure of ASD


Amplatzer FDA approved, over 9,000 used with excellent results

Murphy JG, et al.


  

123 pt Mayo Clinic 1956-1960 ASD repair 1956

62% female, mean age 26 (2-62) (2-

 

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)

Mortality followup at 27 years


Age Repair Age/Sex Matched Control <25 93% 97% 25-40 2584% 91% >41 40% 59%

Survival Curves

Murphy JG, et al - Summary




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

82 pt (34 med 48 surgical)




70% asymptomatic, Mean PAP sys 34/30

 

25 year followup Outcome measures




Survival , symptoms, and complications

Outcomes/Follow-up at 25 years Outcomes/FollowMedical (34)


Presentation Follow up

Surgical (48)
Presentation Follow up

CV Death NYHA I NYHA II NYHA III Atrial Fibrillation 25 (74%) 9 (26%) 0 (0%) 7 (20%)

1 (3%) 19 (56%) 15 (44%) 0 (0%) 19 (56%) 34 (71%) 14 (29%) 0 (0%) 12 (25%)

2 (4%) 26 (54%) 22 (46%) 0 (0%) 28 (53%)

Shah, et al. Conclusions




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%

Clinical / Baseline characteristics

PVR, Qp/Qs Med Rx included Dig, diuretics or nitrates 94% of pt symptomatic

Results
Medical
10yr Surv. NYHA worse NYHA better1 Afib/flutter
169%

Surgery
95% p=.02 11% 32% 15%

84% 34% 3% 17%

improvement in NYHA III/IV

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

Historical Perspectives - 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
 

1/3 of all Adult congenital heart disease 2-3:1 female to male

ASD - Anatomy
Ostium Secundum -75% Ostium Primum - 15% Sinus Venosus - 10%

Braunwauld 6th ed

Associated conditions/ECG abnormalities




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
  

Size of defect Relative compliance of ventricles Relative resistance of pulmonary/systemic circulation

  

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

Atrial Septal Defect


 

1/1500 live births Secundum


 

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


Coronary sinus (rare)




ASDASD- Anatomy/Prevalence
Secundum 75% Primum 15% Sinus Venosus 10% Cor Sinus (rare)

Braunwaulds Heart Disease, 6th ed

ASD - Clinical


Majority repaired in childhood, but may present in adolescence/adulthood Asymptomatic




murmur, abnl ECG/CXR dyspnea/CHF CVA/emboli Atrial Fibrillation

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
 

Oximetry Shunt Ratio (Qp/Qs)

Grossman, Cardiac Cath. 6th ed Ch 9

Catheterization/Oximetry

Grossman; Keane JF et al, Grossman Cardiac Cath.6th ed Chs 9,34

Treatment
 

Medical : diuretics, ACEI, Aldactone Repair


Consider when sxs, Qp:Qs>1.5  Surgical


Mortality 1-3% in most series 1 PVR > 6-8 Woods Units - Contraindication 6

Interventional
Only for secundum defects  94-96% success (Amplatzer) 94

Percutaneous Devices used for Closure of ASD


Amplatzer FDA approved, over 9,000 used with excellent results

Early Studies of Prognosis/Natural History


 

1941 Bedford describes clinical features 1 1957, 1970 Campbell 2,3




untreated mortality


25% Age 30, 75% age 50, 90% age 60

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,

Murphy JG, et al.


  

123 pt Mayo Clinic 1956-1960 ASD repair 1956

62% female, mean age 26 (2-62) (2-

 

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)

Mortality followup at 27 years


Age Repair Age/Sex Matched Control <25 93% 97% 25-40 2584% 91% >41 40% 59%

Survival Curves

Murphy JG, et al - Summary




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

82 pt (34 med 48 surgical)




70% asymptomatic, Mean PAP sys 34/30

 

25 year followup Outcome measures




Survival , symptoms, and complications

Outcomes/Follow-up at 25 years Outcomes/FollowMedical (34)


Presentation Follow up

Surgical (48)
Presentation Follow up

CV Death NYHA I NYHA II NYHA III Atrial Fibrillation 25 (74%) 9 (26%) 0 (0%) 7 (20%)

1 (3%) 19 (56%) 15 (44%) 0 (0%) 19 (56%) 34 (71%) 14 (29%) 0 (0%) 12 (25%)

2 (4%) 26 (54%) 22 (46%) 0 (0%) 28 (53%)

Shah, et al. Conclusions




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%

Clinical / Baseline characteristics

PVR, Qp/Qs Med Rx included Dig, diuretics or nitrates 94% of pt symptomatic

Results
Medical
10yr Surv. NYHA worse NYHA better1 Afib/flutter
169%

Surgery
95% p=.02 11% 32% 15%

84% 34% 3% 17%

improvement in NYHA III/IV

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

Gilkeson, R. C. et al. Am. J. Roentgenol. 2003;180:973-980

Copyright 2007 by the American Roentgen Ray Society

--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

Gilkeson, R. C. et al. Am. J. Roentgenol. 2003;180:973-980

Copyright 2007 by the American Roentgen Ray Society

--1-week-old boy with hypoplastic left heart syndrome

Gilkeson, R. C. et al. Am. J. Roentgenol. 2003;180:973-980

Copyright 2007 by the American Roentgen Ray Society

--23-year-old woman with tricuspid atresia after insertion of Glenn and Fontan shunts who presented with new atrial fibrillation

Gilkeson, R. C. et al. Am. J. Roentgenol. 2003;180:973-980

Copyright 2007 by the American Roentgen Ray Society

Etiologic Basis of Congenital Heart Diseases


1. Primary genetic factors (10%) 1) Chromosomal ; 5-10% 2) Single mutant gene ; 3% Recessive Dominant 2. Genetic-environmental interaction Genetic(90%) 1) Multifactorial inheritance ; majority 2) Risks to offspring of an affected parent 3) Environmental contribution Drugs Infections Maternal conditions

Potential Cardiovascular Teratogens


2. Infections
Rubella Coxsakie virus

1. Drugs
Alcohol Amphetamines Anticonvulsants Chemotherapy Sex hormone Thalidomide Retinoic acid

3. Maternal conditions Old age


Diabetes Lupus Phenylketonuria

4. Others

Maternal Risk Factors




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)

Maternal Drug Exposures



      

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

Stages of Heart Formation


1. Early blood vessel formation
1) Intraembryonic blood vessel at 13 days 2) Extraembryonic blood vessels at 17 days

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

Incidence of Congenital Heart Diseases


1.

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 %

Evaluation of CHD by History Taking


1. Infants
1) Murmur 2) Symptoms of CHF poor feeding, low weight gain, tachypnea, tachycardia, sweating, anxiety, irritability, frequent URI 3) Symptoms of hypoxemia cyanosis, hypoxic spell

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

To Be Corrected in Infancy & Childhood


           

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

VSD PS Mitral stenosis Atrial isomerism

Double Inlet Ventricle


A cardiac anomaly in which both atria connect to only one ventricular chamber by either two separate AV valve or a common AV valve

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

Anatomically Corrected Malposition of Great Arteries


A cardiac anomaly in the position of the great arteries and not in cardiac connection. The LV probably always exhibits a well formed subaortic conus and the RV has less well developed infundibulum than normal
 

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

Double Outlet Left Ventricle


A cardiac anomaly in which both great arteries arise from the left ventricle with nearly always with large VSD, and frequent occurrence of PS. Dx is an indication for op. Without PS : Performed in the 1st 6mo of life Banding and delayed repair Intracardiac baffling Conduit repair With PS : Classic shunt before the age of 2 years Simple intraventricular repair LeCompte procedure in infancy Conduit repair in childhood

Anomalous LCA From the PA


Whole of the left main or LAD, or circumflex branch connects anomalously to main pulmonary artery or rarely from proximal RPA. Very rarely, both coronary arteries connect to the pulmonary artery by a single trunk. Urgent operation is indicated in infancy. The condition is an also indication for op in older patient as well. Construction of a two coronary system LCA transfer CABG SubclavianSubclavian-left coronary anastomosis Tunnel operation Ligation of LCA

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

Gilkeson, R. C. et al. Am. J. Roentgenol. 2003;180:973-980

Copyright 2007 by the American Roentgen Ray Society

--23-year-old woman with Down syndrome and history of atrial ventricular canal who presented with new onset of chest pain

Gilkeson, R. C. et al. Am. J. Roentgenol. 2003;180:973-980

Copyright 2007 by the American Roentgen Ray Society