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May 2011 Peerapat Thaisiam Yossavadee Ruamcharoen

Incidence of moderate and severe forms of CHD is 6 per 1000 livebirths Include bicuspid aortic valves -> 19 per 1000 livebirths Without early medical or surgical treatment, the majority of patients with complex CHD would not survive to adulthood Result from altered embryonic development of a normal structure

Gender preponderance PDA, Ebstein anomaly, ASD : more common in females Aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome, pulmonary and tricuspid atresia, and TGA : more common in males

Extracardiac anomalies occur in about 25%

Genetic disorder Single gene mutation : familial forms of ASD with prolonged AV conduction; mitral valve prolapse; VSD; congenital heart block; situs inversus; pulmonary hypertension; and the syndromes of Noonan, LEOPARD, Ellisvan Creveld, and Kartagener Several defects : long-QT syndrome, Holt-Oram syndrome, Marfan syndrome, HCM supravalvular aortic stenosis <15% of all cardiac malformations : chromosomal aberrations or genetic mutations or transmission Maternal rubella, thalidomide and isotretinoin early during gestation, and chronic maternal alcohol abuse

Environmental toxin

Multifactorial genetic and environmental

 Hemodynamics - CHF Cyanosis Pulmonary hypertension - Eisenmenger Infective endocarditis Cardiac arrhythmia - asso previously Pregnancy
repaired CHD

Progressive myocardial dysfunction : pressure or volume overload Present with left, right, biventricular, or univentricular failure Intrinsic myocardial abnormalities : restrictive diastolic Chronic low cardiac output state, Volume overload, Congestive hepatopathy, ascites, and protein losing enteropathy Presence of a substrate : myocardial dysfunction, valvular regurgitation Precipitant : sustained arrhythmia, pregnancy, hyperthyroidism

CHF is not common in adult congenital heart

Reninangiotensin system : NOT play a role

Arterial oxygen desaturation : shunting or mixing of systemic venous blood into the arterial circulation

Clinical features Hyperviscosity syndrome Hematologic Bleeding tendency : Elevated prothrombin and partial thromboplastin times, decreased factor levels (factors V, VII, VIII, and IX), qualitative and quantitative platelet disorders, increased brinolysis, and systemic endothelial dysfunction

Neurological : cerebral hemorrhage, paradoxical emboli Renal : proteinuria, hyperuricemia, or renal failure Rheumatologic : hypertrophic osteoarthropathy

 Pulmonary vascular obstructive disease :

Exercise intolerance 3rd decade : slowly progressive decline in Most common modes of death are sudden
death, CHF , and pulmonary hemorrhage

consequence of a large preexisting left-to-right shunt

After 40 yr : CHF

their physical abilities

HIGH risk : prosthetic valves, cyanosis, systemic or pulmonary artery conduits, and previous endocarditis

MODERATE risk : other CHD LOW risk surgical repair of ASD , VSD , PDA (without residua > 6 months

Only mod-to-high risk : need ATB prophylaxis for dental, respiratory, genitourinary, or gastrointestinal procedures

 Arrhythmias and conduction defects SND : extensive atrial reconstruction Atrial tachyarrhythmia :

Ventricular tachyarrhythmia : after TOF repair

Atrioventricular valve disease or shunts Scar-mediated reentrant atrial utter

Pregnancy counseling is mandatory for all patients Obstructive lesions are less well tolerated than regurgitant lesions or shunts Heart failure and arrhythmia are major complications The incidence of early spontaneous abortion is proportional to the severity of cyanosis Tissue abnormalities of the aorta : increase risk rupture or dissection Prosthetic valves : teratogenic Vaginal delivery

 Left to right shunt Atrial septal defects Patent foramen ovale Atrioventricular septal defect Ventricular septal defect Patent ductus arteriosus Cyanotic heart disease Tetralogy of Fallot Tricuspid Atresia Hypoplastic Left Heart Syndrome Double-Inlet Left Ventricle Total Anomalous Pulmonary Venous Connection Transposition Complexes Ebstein Anomaly LVOT Coarctation of the aorta

Ostium secundum defects

Excessive resorption of the septum primum or Decient growth of the septum secundum

Sinus venosusIVC defects are very uncommon PATHOPHYSIOLOGY

Degree of shunting : size of the defect and the relative diastolic lling 2 ventricles Increase shunt : any condition causing

reduced left ventricular compliance (e.g., systemic hypertension, car- diomyopathy, myocardial infarction) or increased left atrial pressure (mitral stenosis or regurgitation)

Natural history

large ASD = Qp/Qs > 2.0:1.0, CHF and FTT sig. ASD = Qp/Qs > 1.5 :1.0 , symptoms in adolescence or adulthood Eort dyspnea : 30% in 3rd decade, > 75% in 5th decade Supraventricular arrhythmias and right-sided HF : 40 years Paradoxical embolism Pulmonary hypertension

Clinical features Exercise intolerance and palpitations (typically from Autter, Abrillation, or SSS) Right ventricular failure : presenting symptom in older Cyanosis : shunt reversal and Eisenmenger syndrome

left atrializationof JVP (A wave = V wave) Hyperdynamic right ventricles Wide and xed split of S2 is hallmark SEM, usually gr. 2 : 2nd left ICS, and a mid-diastolic rumble LLPSB, from increased ow through the tricuspid valve, PSM of TR : Rt. ventricular failure ELECTROCARDIOGRAPHY.

Sinus rhythm or Autter or Abrillation The QRS axis is typically rightward in secundum ASD Negative P waves in the inferior leads -> low atrial pacemaker -> sinus venosus superior vena cava defects Complete RBBB Tall R or R waves in V1 often indicate pulmonary hypertension.

CHEST RADIOGRAPHY. Cardiomegaly RAE , RVH Prominent pulmonary trunk and increase pulmonary ow Small aortic knuckle

Presence of right-sided heart dilation and a signicant ASD (>5 mm) Qp/Qs < 1.5 do not require closure, with the possible exception of trying to prevent paradoxical emboli in older patients after a stroke Signicant ASDs (Qp/Qs > 1.5,or ASDs associated with right ventricular volume overload) pulmonary hypertension : net left-to-right shunt of at least 1.5:1 or evidence of pulmonary artery reactivity on challenge with a pulmonary vasodilator

DEVICE CLOSURE only for secundum ASD with a stretched diameter of <41 mm and with adequate rims to enable secure deployment of the device major complications <1% and clinical closure achieved in >90% sinus venosus or ostium primum defects secundum defects with unsuitable anatomy

SURGERY

After device closure, 6 mo of aspirin and endocarditis prophylaxis

Tunnel-like space between the overlying septum secundum and septum primum Typically closes in 75% at birth Associated with

atrial septal aneurysms eustachian valves (a remnant of the sinus venosus valve) Chiari networks (lamentous strands in the right atrium)

Conduit for paradoxical embolization Stagnant ow, nidus for in situ thrombus formation Cryptogenic stroke , the usual causes of stroke must rst be eliminated Diagnosis by TTE, TEE -> microbubbles are seen in the left-sided THERAPY : antiplatelet or anticoagulant agents, percutaneous device closure and surgical PFO closure

atrioventricular canal defect endocardial cushion defect

Spectrum of lesions involving

atrial and ventricular septum atrioventricular valves left ventricular outow tract

"partial" form = primum ASD but no VSD "complete" = primum ASD and an inlet VSD Deciency of the inlet ventricular septum + abnormalities of the AV valves -> elongated LVOT "goose neck" Subaortic stenosis : chordal attachments of the cleft anterior mitral valve to the left ventricular outow septum

Isolated primum ASD ~ large secundum ASDs

+/- signicant left AV valve regurgitation Asymptomatic until 30-40 yr, CHF, atrial arrhythmias, CHB, and pulmonary hypertension

Complete AV canal ~ size of ASD and VSD, AV valves Large left- to-right shunt -> heart failure or pulmonary vascular disease, palpitations from atrial arrhythmias 35% of Downs syndrome : AV canal defect (more complete)

Echo : 4-chamber view, the AV valves appear at the same level

Unrepaired or newly diagnosed AV septal defect and signicant hemody- namic defects Persistent left AV valve regurgitation causing symptoms Signicant subaortic obstruction (mean gradient >50 mm Hg at rest) In case of severe PHT, net left-to-right shunt of >1.5 :1 or evidence of pulmonary artery reactivity on challenge with a pulmonary vasodilator ASD primum -> Pericardial patch closure with suture cleft left AV valve +/- MVR Complete -> primary intracardiac repair in infancy > staged approach

Restrictive VSD

pulmonary-to-aortic systolic pressure ratio < 0.3 Qp/Qs 1.4 : 1

Moderately restrictive VSD pulmonary-to-aortic systolic pressure ratio < 0.66 Qp/Qs of 1.4 to 2.2:1

Large or non- restrictive VSD pulmonary-to-aortic systolic pressure ratio > 0.66 Qp/Qs > 2.2 Cyanosis in adult Eisenmenger, sign of PHT, clubbing of nger

Not cause signicant hemodynamic derangement May close spontaneously during childhood and sometimes in adult life A perimembranous defect in an immediately subaortic position ~ progressive aortic regurgitation Asymptomatic Harsh or high-frequency PSM, usually grade 3 to 4/6, heard at the left sternal border 3rd - 4th intercostal space ECG - normal tracing Treatment : IE prophylaxis , follow up BUT...Surgery in Small defects with aortic valve prolapse and AR

Hemodynamic burden LV -> LAE, LVH and dysfunction Increase in pulmonary vascular resistance ECG : notched P wave , left ventricular volume overload, deep Q and tall R with tall T in V5 and V6

Indicate surgery symptomatic patient Qp/Qs > 1.5 :1, pulmonary artery systolic pressure > 50 mm Hg, increased left ventricular and left atrial size, or deteriorating left ventricular function absence of irreversible pulmonary hypertension

Direct suture with patch

The ductus arteriosus ~ Lt. 6th primitive aortic arch and connects the proximal left pulmonary artery to the descending aorta, just distal to the left subclavian artery Silent : tiny PDA detected only by nonclinical means (usually echocardiography) Small : continuous murmur common; Qp/Qs < 1.5 : 1 Moderate : continuous murmur common; Qp/Qs of 1.5 to 2.2 : 1 Large : continuous murmur present; Qp/Qs > 2.2 : 1 Eisenmenger: continuous murmur absent; substantial pulmonary hypertension, dierential hypoxemia, and dierential cyanosis (pink ngers, blue toes)

Silent PDA : no long term complication Small ductus may predispose to endarteritis Moderate-sized duct and shunt pose a volume load on LA and LV , Abrillation Large duct results initially in left ventricular volume overload but develops a progressive rise in pulmonary artery pressures machinery murmur -> wide systemic pulse pressure from aortic diastolic runo into the pulmonary trunk ECG : moderate duct show left ventricular volume overload CXR : moderate-sized duct , cardiomegaly with left-sided heart enlargement, a prominent aortic knuckle, and increased pulmo- nary perfusion

 INTERVENTION Hemodynamic signicant Debate in inaudible or small PDA

strictly to reduce the risk of endarteritis

Transcatheter treatment :ducts smaller Surgical : ductal ligation, too large for
device closure

 Double-outlet right ventricle (aortic overrid > 50%) May coexist with an AV septal defect RVOT is variable : atresia, stenotic, bicuspid

pulmonary valve with supravalvular hypoplasia exists The dominant site of obstruction is usually at the subvalve level Major aortopulmonary collateral arteries

 Right aortic arch Abnormalities of the course of the coronary arteries :

the anterior descending artery originates from the right coronary artery Absent pulmonary valve syndrome The pulmonary arteries are usually markedly dilated or aneurysmal

 cyanosis paroxysmal hypoxemia right ventricular impulse systolic thrill are often palpable along the LSB early systolic ejection sound at the lower LSB and apex the second heart sound is usually single the intensity and duration of the systolic ejection murmur

vary inversely with the severity of subvalve obstruction continuous murmur faintly audible over the anterior or posterior chest reects ow through aortopulmonary collateral vessels or a duct.

 EKG : RAD, RAE, RVH, RBBB [post repair], QRS width

may reect the degree of right ventricular dilation >> may be a risk factor for sustained VT and sudden death CXR : a normal-sized, bootshaped heart with prominence of the right ventricle and pulmonary vascular markings are diminished, and the aortic arch may be on the right side, ascending aorta is prominent Echocardiography :

 systemic to pulmonary shunt : MBTS Surgical repair

 Absence of the right AV connection must be an ASD hypoplasia of the morphologic right ventricle which

communicates to the dominant ventricle via a VSD subdivided into those with concordant ventriculoarterial connections and normally related great arteries (70% to 80% of cases) and those with discordant connections

 subaortic stenosis and aortic arch anomalies

[discordant]

 All patients have mixing of atrial blood concordant ventriculoarterial connections tend to be

more cyanosed (depending on the size of the VSD) discordant connections are pinker and tend to develop heart failure (because the unobstructed pulmonary circulation arises directly from the left ventricle

 EKG : LAD, RAE, LVH CXR : situs solitus, levocardia and a left-sided aortic

arch, the heart size and pulmonary vascular markings vary with the amount of pulmonary blood ow Echocardiography :

 Concordant : systemic to pulmonary shunt

[bidirectional Glenn procedure] Discordant : pulmonary artery banding to reduce pulmonary blood ow

 underdevelopment of the left cardiac chambers atresia or stenosis of the aortic or the mitral orice

and hypoplasia of the aorta Duct dependent systemic blood ow

 Any infant with the sudden onset of circulatory

collapse and severe lactic acidosis

 EKG RAD, RAE, RVH, ST and T wave abnormalities in

the left precordial leads CXR : cardiomegaly and increased pulmonary venous and arterial vascular markings Echocardiography :

 Early treatment with prostaglandin Staged surgical management now provides long-term

palliation The survivors of the earliest attempts at staged Norwood palliation are just now entering adult life

 the dominant ventricle is a left ventricle [75%] separated from the right ventricle by a VSD ventriculoarterial connection is discordant the aorta arises from the small right ventricle and is

fed via the VSD unobstructed pulmonary artery arises from the left ventricle Subaortic stenosis, aortic hypoplasia, and arch anomalies are common

 severe cyanosis or with duct-dependent pulmonary

blood ow heart failure because of increased pulmonary blood ow

 EKG : variable CXR : variable Echocardiography :

 surgical intervention to establish the pulmonary valve

as the unobstructed systemic outow tract Pulmonary artery banding is oered only to those infants with pulmonary overcirculation, heart failure, and unobstructed systemic outow

 situation in which all pulmonary veins fail to connect

directly to the morphologic left atrium all of the systemic and pulmonary venous return usually drains to the right atrium subdivided according to the path of the abnormal drainage connecting via a vertical vein to the left brachiocephalic vein, directly to the right atrium, directly to the superior vena cava, below the diaphragm [10%]

 signs of heart failure or cyanosis clinical picture depend on the size of the interatrial

communication and the degree of obstruction a xed, widely split second heart sound with an accentuated pulmonic component

 EKG : RAD, RAE, RVH CXR : cardiomegaly [RA, RV] with increased

pulmonary blood ow, pulmonary a. segment is enlarged. The so-called gure-of-8 or snowman heart is due to enlargement of the heart and the presence of a dilated right superior vena cava, innominate vein, and left vertical vein Echocardiography :

 balloon atrial septostomy is occasionally required to

increase systemic blood ow before surgery Surgical repair

 ventriculoarterial discordance
AV concordance : complete TGA or D-TGA AV discordance : congenitally corrected TGA or L-TGA

clinical manifestations are dyspnea and cyanosis

from birth, progressive hypoxemia, and congestive heart failure

 The origin of the aorta from the morphologic right

ventricle and that of the pulmonary artery from the morphologic left ventricle communication between the two circulations must exist after birth to sustain life : ASD, VSD, PDA

 EKG : variable CXR : a narrow vascular pedicle with an oblong

cardiac silhouette (egg on side) is typically seen in patients after the atrial switch procedure. Echocardiography :

 balloon atrial septostomy Surgical repair

 systemic venous blood passes from the right atrium

through a mitral valve to a left ventricle and then to the posteriorly located pulmonary artery Pulmonary venous blood passes from the left atrium through a tricuspid valve to a left-sided right ventricle and then to an anterior, left sided aorta Associated anomalies : VSD (75%), pulmonary or subpulmonary stenosis (75%), and left-sided tricuspid and often Ebstein-like) valve anomalies (>75%) inherently abnormal conduction system : complete heart block

 Asymptomatic Dyspnea, exercise intolerance from developing

congestive heart failure palpitations from supraventricular arrhythmias VSD & PS : emboli or cyanosis The A2 is often palpable in the second Lt. ICS A single S2 (A2) is heard, with P2 often being silent because of its posterior location murmur of an associated VSD or of left AR may be heard

 EKG : reversal of the precordial Q wave pattern, 1st-

degree AV block, complete AV block, atrial arrhythmias CXR : convexity of the left supracardiac border produced by the left-sided ascending aorta, main pulmonary trunk is medially displaced and absent from the cardiac silhouette, right pulmonary hilum is often prominent and elevated compared with the left, producing a right-sided waterfall appearance. Echocardiography :

 Medical therapy : ACE inhibitor or beta blocker

therapy for patients with systemic ventricular dysfunction Surgical repair

 apical displacement of the septal tricuspid leaet in

conjunction with leaet dysplasia atrialization (functioning as an atrial chamber) of the inow tract of the right ventricle and consequently produces a variably small functional right ventricle Associated anomalies include PFO or ASD, accessory conduction pathways, RVOT, VSD, aortic coarctation, PDA, or mitral valve disease tricuspid regurgitation, right atrial enlargement Right-to-left shunting through a PFO or ASD

 exercise intolerance palpitations of supraventricular origin cyanosis from a right-to-left shunt at atrial level right sided cardiac failure from severe TR and right

ventricular dysfunction unimpressive jugular venous pressure widely split S1 with a loud tricuspid component widely split S2 from a RBBB, and a right-sided S3 PSM increasing on inspiration from TR is best heard at the LLPSB

 EKG : RAD, RAE, AV block, RBBB, AF/utter CXR : rightward convexity from an enlarged right

atrium and atrialized right ventricle coupled with a leftward convexity from a dilated infundibulum gives the heart a water bottle appearance, pulmonary vasculature is usually normal to reduced Echocardiography :

 substantial cyanosis right-sided heart failure poor functional capacity paradoxical emboli recurrent supraventricular arrhythmias [uncontrol] asymptomatic substantial cardiomegaly

(cardiothoracic ratio > 65%)

 discrete narrowing at the ligamentum arteriosum diuse forms may involve the arch or isthmus descending aorta distal to the segment of coarctation

is often aneurysmal intracranial aneurysms [10%] systemic arterial hypertension in the upper extremities association with gonadal dysgenesis (Turner syndrome) and bicuspid aortic valve, VSD, MR, MS

 EKG : RAD, RVH CXR : cardiomegaly and pulmonary arterial and

venous engorgement, rib notching

 Excision of the narrowed segment and end-to-end

anastomosis prosthetic grafts Percutaneous balloon angioplasty with stenting