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Non-cyanotic Congenital Heart Disease Medical Cadet Weerayut Wiriyabanditkul Non-cyanotic Non-cyanotic congenital heart disease congenital heart disease

Non-Cyanotic Congenital Heart Disease-UpDate 4-22-53

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Page 1: Non-Cyanotic Congenital Heart Disease-UpDate 4-22-53

Non-cyanotic Congenital Heart Disease

Medical Cadet Weerayut Wiriyabanditkul

Non-cyanotic Non-cyanotic congenital heart diseasecongenital heart disease

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Outline

Non-cyanotic congenital heart disease1. Lesions Resulting Normal Pulmonary Blood Flow2. Lesions Resulting Increased Pulmonary Blood Flow3. Other Anomalies

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Non-cyanotic Congenital Heart Disease

1. Lesions Resulting Normal Pulmonary Blood Flow1. Congenital valvar aortic stenosis2. Coarctation of aorta3. Cor Triatriatum

2. Lesions Resulting Increased Pulmonary Blood Flow1. Ventricular septal defect2. Atrial septal defect3. Patent ductus arteriosus4. Rupture aneurysm of sinus of valsava5. Interrupted of aortic arch6. Truncus arteriosus

3. Other Anomalies1. Vascular ring2. Vascular sling

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VSD

ASD

PDA

Rupture anuerysm of sinus of valsava

IAA

TA

1. Lesions Resulting Increased Pulmonary Blood Flow

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Coarctation of aorta

Cor tratraitumCongenital aortic stenosis

2. Lesions Resulting Normal Pulmonary Blood Flow

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3. Other Anomalies

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1.Lesions Resulting Increased Pulmonary Blood Flow

1. Ventricular septal defect2. Atrial septal defect3. Patent ductus arteriosus4. Rupture aneurysm of sinus of valsava5. Interrupted of aortic arch6. Truncus arteriosus

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1. Ventricular Septal Defect

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Definition

• Hole between left and right ventricle• Most common congenital heart disease 30-

40%• Most common type is Membranous type• But Perimembranous type 80% Surgery

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Ventricular Septal Defect

• magnitude of the shunt is determined by– the size, not the location – the level of pulmonary vascular resistance

(PVR).

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Ventricular Septal Defect

• Small defect a large resistance at defect– shunt does not depend on the level of PVR.

• With a large VSD resistance is minimal – shunt depends on the level of PVR. – The lower the PVR the magnitude of shunt↑.

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• enlargement of the main PA, LA, and LV, as well as an increase in pulmonary vascular markings

Pansystolic

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• Enlargement of the LA, LV, PA and increased pulmonary vascular markings

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The difference between VSD and ASD

- presence of left atrial enlargement – The similarities between VSD and patent ductus

arteriosus (PDA) : an enlarged LA and LV

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Ventricular Septal Defect

• most common form of congenital heart defect

• ventricular septum divided into – a small membranous portion – a large muscular portion : three components—

the inlet septum, the trabecular septum, and the outlet septum

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Supracristal/Infundibular type

Muscular VSD

Perimembranous VSD

Atrioventricular canal

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Epidemiology

• 20-30%• 80% of perimembranous type require surgery.

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CLINICAL MANIFESTATIONS

History 1.small VSD asymptomatic with normal growth and

development 2.moderate to large VSD– delayed growth and development– decreased exercise tolerance– repeated pulmonary infections– CHF :common during infancy

3.long-standing pulmonary hypertension history of cyanosis + decreased level of activity

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Physical Examination

1.small VSDs acyanotic.

large VSDs poor weight gain or signs of CHF. Eisenmenger's syndrome Cyanosis and clubbing

2. systolic thrill may be present at the lower left sternal border.

Precordial bulge and hyperactivity are present with a large-shunt VSD.

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NATURAL HISTORY

1.Spontaneous closure occurs in 30% to 40% with membranous VSDs and muscular VSDs during the first 6 months of life.

2.CHF develops in infants with large VSDs, but usually not until 6 to 8 weeks of age.

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NATURAL HISTORY

3.Pulmonary vascular obstructive disease may begin to develop as early as 6 to 12 months

resulting right-to-left shunt usually does not develop until the teenage years.

4. Infective endocarditis rarely occurs.

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X-ray Studies

1.Cardiomegaly LA, LV, Pulmonary vascular markings increase.

2.In pulmonary vascular obstructive disease, PA and the hilar PAs enlarge heart size is usually normal.

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Echocardiogram

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MANAGEMENT

Medical 1.Treatment of CHF, if it develops, is indicated

with digoxin and diuretics2.No exercise restriction is required in the

absence of pulmonary hypertension. 3.Maintenance of good dental hygiene and

antibiotic prophylaxis against infective endocarditis

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SurgicalIndications and Timing• Small infants who have large VSDs and develop CHF

and growth retardation are first Manage with digoxin and diuretics.

• If growth failure cannot be improved by medical therapy

• Older infants with large VSDs + evidence of elevated pulmonary vascular resistance should be operated on as soon as possible

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Procedure• Requiring use of CPB + mod. Hypothermia +

cardioplegia• Patch repair through right atrial approach. (minimally

invasive surgical techniques with smaller skin incisions ) Cosmetic

• Left ventriculotomy in apical muscular VSD.• Percutaneous Amplatzer device

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Percutaneous Amplatzer device

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2. Atrial Septal Defect

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Definition

• Opening in the interatrial septum that enable themixing of blood from systemic and pulmonary venous

• 10-15% of all congenital heart disease• Most common type is Ostium secundum 80%• More common in females

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PATHOLOGY

• There are three types of ASDs1. Ostium secundum defect is the most

common type of ASD(50%-70% )2. Ostium primum defects (30%)3. Sinus venosus defect ( 10%)

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Atrial Septal Defect

• magnitude of the left-to-right shunt is determined by – size of the defect – compliance of RV and LV

• The compliance of RV > LV left-to-right shunt

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• the RA, RV, and main PA and its branches have two arrows dilated

Systolic ejection

Wide and fixed split S2

Large defectRelative TS

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CLINICAL MANIFESTATIONS

History• Infants and children with ASDs are usually

asymptomatic.

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CLINICAL MANIFESTATIONS

Physical Examination 1. A relatively slender body build is typical. 2. A widely split and fixed S2 and a grade 2-3/6 systolic ejection murmur. With a large left-to-right shunt, a mid-diastolic rumble resulting from relative tricuspid stenosis at the lower left sternal border. 3. The typical auscultatory findings may be absent in infants

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X-ray Studies

1. Cardiomegaly with enlargement of the RA and RV is present.

2. A prominent pulmonary artery segment and increased pulmonary vascular markings can be seen.

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• CXR : reveal enlargement of the RA, RV, and PA, as well as an increase in pulmonary vascular markings

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• The heart is mildly enlarged, with involvement of the right atrium (best seen in the posteroanterior view) and the right ventricle (best seen in the lateral view with obliteration of the retrosternal space). Pulmonary vascularity is increased, and the main pulmonary artery segment is slightly prominent.

RV enlargement

RA enlargement

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Atrial Septal Defect

• Absence of left atrial enlargement is one of the helpful x-ray signs for differentiating an ASD from VSD

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Electrocardiography

• Right axis deviation • mild right ventricular hypertrophy (RVH)

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Surgical ClosureIndications

- A left-to-right shunt with a pulmonary-to-systemic blood flow ratio ( p/ s) of ≥1.5:1 indicates the need for surgical closure. - a smaller shunt to be a surgical indication because of the risk of paradoxical embolization and cerebrovascular accident.

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Surgical Closure

Timing. - Surgery is usually delayed until the patient is 3 to 4 years of age because the possibility of spontaneous closure exists.

- surgery is performed during infancy if CHF does not respond to medical management

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Surgical Closure

• Procedure. • a midsternal incision under cardiopulmonary

bypass by either a simple suture or a pericardial or Teflon patch.

• Recently, so-called minimally invasive cardiac surgical techniques with smaller skin incisions have become popular, especially for female patients.

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3.Patent Ductus Arteriosus

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Definition

• Connection (patent) from main or left pulmonary artery to upper descending thoracic aorta

• 1:2000 births• Increase dramaticlly in prematurity• Female > male 2:1• PDA usually closed in 48 hours after birth.

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Upper descending thoracic aorta connect with Proximal part of pulmonary artery

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Patent Ductus Arteriosus

• Hemodynamics of PDA are similar to VSD • left-to-right shunt is determined

Ductus is small– the resistance by the ductus (diameter, length,

and tortuosity) Ductus is large– level of PVR

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Patent Ductus Arteriosus

• 5% - 10% of all congenital heart defectsPATHOLOGY 1.There is a persistent patency of a normal

fetal structure between the left PA and the descending aorta,

2.The ductus is usually cone shaped with a small orifice to the PA

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CLINICAL MANIFESTATIONS

History 1.usually asymptomatic 2.lower respiratory tract infection, atelectasis

and CHF (accompanied by tachypnea and poor weight gain)

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Physical Examination

1.Tachycardia and exertional dyspnea. With pulmonary vascular obstructive disease, a right-to-left ductal shunt results in cyanosis only in the lower half of the body

2.The precordium is hyperactive. A systolic thrill may be present at the upper left sternal border. Bounding peripheral pulses with wide pulse pressure.

3.The P2 is usually normal, A grade 1-4/6 continuous (“machinery”) murmur is best audible at the left infraclavicular area

4. Elevated jugular venous pressure and Liver enlargement.

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Electrocardiography.

• similar to those for VSD• LVH is seen with small to moderate PDA. • CVH(Combined ventricular hypertrophy) is

seen with large PDA. If pulmonary vascular obstructive disease develops, RVH may be seen.

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X-ray Studies.

• similar to those of VSD. 1.normal with a small-shunt PDA. 2.Cardiomegaly of varying degrees occurs with enlargement of

the LA, LV, and ascending aorta. Pulmonary vascular markings are increased.

3.With pulmonary vascular obstructive disease, the heart size is normal, with a marked prominence of the PA segment and hilar vessels.

CXR of PDA are indistinguishable from VSD.

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MANAGEMENT

Medical 1. Indomethacin is effective in premature infant

Inhibition of Prostaglandin synthesisIndomethacin is ineffective in term infants with PDA and should not be used.

2.No exercise restriction is needed (absence of pulmonary hypertension)

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Nonsurgical Closure.

• Transluminal placement of various occlusive devices. (PDA<3cm.) (better in older than age5)

The advantages – no need for general anesthesia– shorter hospital stay and convalescent period, – elimination of a thoracotomy scar.

Disadvantages and potential complications– peripheral coil embolization, – left PA stenosis, and femoral vessel occlusion

These techniques may not be applicable in very young infants.

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Surgical Closure

Indications1. Premature baby with CHF and Medication failure or

contraindicated with medication(Such as azothemia, evidence of bowel ischemia, thrombocytopenia, intracerebral or other hemorrhage and sepsis.)

2. Size of PDA >=3 cm • The presence of pulmonary vascular obstructive

disease is a contraindication to surgery.

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Procedure

• Standard procedure– Triple ligation of the ductus with permanent suture

through left posterolateral thoracotomy without cardiopulmonary bypass

– Patch closure– Division and oversewing

• Recently, a smaller incision with video-assisted thoracoscopy

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Triple ligation

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Division and oversewing

Patch closure

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Prognosis

Mortality ratePremature : very lowOlder infants and children : less than 1%Bleeding, chylothorax, vocal cord paralysis that

need reopened is uncommon.

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Thank youThank you

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4. Ruptured aneurysm of sinus valsava

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Definition

• Congenital aneurysm of the sinuses of Valsava• Common at right sinus through the middle of

noncoronary sinus]• May perforate into the right heart chamber• Aortocardiac fistula

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Clinical features

• Signs and symptoms– Rupture of the sinus aneurysm 35% acute

symptom• Sudden breathlessness• Precordial pain

– Predisposing factor to rupture• Heavy exertion• Bacterial endocarditis• Marfan’s syndrome

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Clinical features

• Examination– Continuous murmur– Widened aortic pulse pressure– Elevated jugular venous pressure with prominent

V wave from tricuspid incompetence

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Investigation

• Electrocardiogram– Left ventricular hypertrophy– May Biventricular hypertrophy

• Chest X-ray– Lung congestion (LeftRight to shunt)– Aortic root not enlargement

• Echocardiogram– Enough for diagnosis

• Cardiac catheterization– Associated anomaly

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Surgery

• Indication– Acute rupture sinus of Valsava Congestive heart failure– Prolong rupture congestive heart failure– Associated anomaly : VSD, aortic incompetence

• Operation– Repair sinus of valsava under cardiopulmonary bypass by

Dacron patch– Closure VSD and Repair aortic incompetence (if there is

associated anomaly)

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5. Interrupted of aortic arch

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Definition

• Interrupted aortic arch is complete luminal and anatomical discontinuity between two segments of the aortic arch. Largeventricular septal defect is nearly always present.

• Rare defect

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Clinical features

• Signs and symptoms– Severe congestive heart failure– Metabolic acidosis– Anuria

• Examination– Diminished femoral pulse– Cardiac murmur

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Investigation

EKG- not specific

Chest X-ray- Gross cardiomegaly and pulmonary congestion

Echocardiography- made for operative intervention- diagnosis

Cardiac catheterization- unsure diagnosis

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Management

PGE1 is infused to maintain ductal patency and correct acidosis

Hemodynamic status should be optimized with mechanical ventilation and inotropic support.

Increase pulmonary resistance Directed blood into systemic circulation by decreasing fractional inspired oxygen and avoiding hyperventilation

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Surgery

Indication- When diagnosis is Interrupted of aortic arch is

indication for surgery.Operation - Complete surgical repair of IAA

Result- Operative mortality is less than 10%

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6. Truncus arteriosus

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Definition

• Rare anomaly• a single arterial conduit that arises from the

heart, overrides the ventricular septum, and supplies the pulmonary, systemic, and coronary circulations.

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Type

• Type I is the same as A1. • Types II and III are grouped as a single type A2

because they are not significantly distinct embryologically or therapeutically.

• Type A3 denotes unilateral pulmonary artery with collateral supply to the contralateral lung (hemitruncus).

• Type A4 is truncus associated with interrupted aortic arch (13% of all cases of truncus arteriosus)

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Clinical features

• Signs and symptoms– Severe congestive heart failure– Cyanosis

• Examination– Systolic murmur or continuous murmur at left

parasternal border

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Investigation

EKG- Bilateral ventricular hypertrophy

Chest X-ray- Gross cardiomegaly and pulmonary congestion

Echocardiography- Definitive diagnosis

Cardiac catheterization- Do in unsure diagnosis

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Surgery

• Indication– When diagnosis Truncus arteriosus is indicationCan control CHF third months of lifeCan not control CHF Immediate surgery

• Operative Technique1.Use cardiopulmonary bypass2.Repair VSD 3.Reconstruction pulmonary a. and aorta by Homograft

valve conduit4.Valve replacement

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Outcome

• Survival rate = 91% in younger than 6 months• Risk factor for poor prognosis and

perioperative death– Severe truncal regurgitation– IAA– Coexistiong coronary anomaly– Age younger than 100 days

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2.Lesions Resulting Normal Pulmonary Blood Flow

1. Congenital valvar aortic stenosis2. Coarctation of aorta3. Cor Triatriatum

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1. Congenital Valvar Aortic Stenosis

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Definition

• Obstruction at valve level caused by imperfect cusp development with leaflet thickening and fusion

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Pathophysiology

– LVOT LVH LA hypertension increased pulmonary vascular resistance

– RVH– Circulatory collapse when PDA closed cyanosis

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Clinical features

• Signs and symptoms– History of poor feeding– Pallor– Perspiration– Dyspnea

• Examination– Systolic ejection murmur– Absent if severe left ventricular failure

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Investigation

• EKG– Left ventricular hypertrophy +/- Right ventricular

hypertrophy• Chest X-ray– Cardiomegaly with pulmonary congestion

• Echocardiogram– Confirm diagnosis and identify other associated anomaly

• Cardiac catheterization– Measurement of systolic gradient to calculate valvular area

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Indication for surgery

Indication1. Transvalvular gradient >50 mmHg +

Symptomatic : syncope, CHF, angina2. Transvalvular gradient 50-75 mmHg + EKG

LV stain and ischemia3. Association defectContraindication : hypoplastic LV

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Surgery

• Indication for surgery– Circulatory collapse from acisosis– Fail medical treatment (Prostaglandin E1)– Fail intervention (Balloon valvuoplasty of aortic

valve)

• Operation– Surgical valvulotomy under cardiopulmonary

bypass

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2. COARCTATION OF THE AORTA

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Definition

• Luminal narrowing of the aorta Obstruction blood flow

• Most commonly located distal to the left subclavian artery

• Extensive collateral intercostals & Mammary arteries

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Pathophysiology

• LV outflow tract obstruction– Pulmonary over circulation– Later Biventricular failure

• Proximal systemic hypertension from– Mechanical obstruction to ventricula ejection– RAAS

• Persistent hypertension• Complication : circle of willis aneurysm, aortic

dissection, risk MI

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Clinical manifestation

• S/S– Left ventricular failure

• Physical examination– Decrease femoral pulse– Hyperdynamic precordium

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Differential cyanosis

cyanosis

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COARCTATION OF THE AORTA

• On chest x-ray films– poststenotic dilatation of the descending aorta

figure-of-3 sign on the plain film – Rib notching

• E-shaped on the barium esophagogram • Echocardiogram– Narrowed aortic segment– Pressure gradient on the stenotic

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COARCTATION OF THE AORTA

• The ECG shows LVH because of a pressure overload on the LV.

• In newborns and small infants, RVH or right bundle branch block (RBBB) is commonly seen, but LVH is not

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Arteriography

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• A, In symptomatic infants, associated defects are frequently found (VSD, aortic and mitral valve abnormalities and hypoplasia of the ascending and transverse aortas ).

• B In asymptomatic children, the coarctation is usually an isolated lesion

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Management

Medical 1.Prostaglandin E1 reopen the ductus arteriosus and

establish flow 2.Intensive anticongestive measures with short-acting

inotropic agents (e.g., dopamine, dobutamine), diuretics, and oxygen

3.Balloon angioplasty useful procedure for sick infants in whom standard surgical management carries a high risk.

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Surgery

• Indication– If diagnosis coarctation is indication for surgery

Procedures.• segment of coarctation is resected • The distal subclavian artery is divided, and the

flap of the proximal portion • Patch aortoplasty Main major complication is aneurysm

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Surgical Procedure

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3. Cor triatriatum

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Definition

• Rare anomaly• the presence of a fibromuscular diaphragm

that partitions the left atrium into two chambers: – Superior chamber : receives drainage from the

pulmonary veins.– Inferior chamber : communicates with the mitral

valve and the left ventricle

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A. Common chamber draining to right atrium directly.B. B. Common chamber draining into systemic venous

circulation via anomalous vein.

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Clinical feature

Signs and symptoms- Low cardiac output : fatigue, dyspnea, decrease peripheral pulse, not growth

Physical examination- loud pulmonary S2 sound

- a right ventricular heave, as well as jugular venous distention and hepatomegaly

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Investigation

• EKG– Right axis deviation, right atrial enlargement and

right ventricular hypertrophy.• Chest x-ray– heart size, pulmonary edema.

• Echocardiogram– Diagnosis

• Cardiac catheterization– If suggest other anomaly.

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Surgery

• Indication– Severe pulmonary edema and pulmonary

hypertension.

• Operation1.Cardiopulmonary bypass and cardioplegic arrest2.Excised membrane between chamber and true left

atrium.

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3. Other anomaly

1. Vascular ring2. Vascular sling

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1. Vascular ring

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Definition

• Abnormal of great vessels(aortic arch and branches) that compress trachea or/and esophagus.

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Double aortic arch

Right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum.

Right aortic arch with mirror-image branching and retroesophageal ligamentum arteriosum.

Left aortic arch with right descending aorta and right ligamentum arteriosum.

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Clinical features

• Signs and symptoms– Difficult to feeding reflux and respiratory

infection– May stridor respiratory distress.

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Investigation

• Chest X-ray– Normal or right aortic arch

• Echocardiogram– Diagnosis vascular ring or other disease.

• CT scan– If + contrast better

• Cardiac catheterization– If others anomaly

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Surgery

• Indication– If significant obstructive symptoms

• Operation– Left thoracotomy– Excision ligamentum arteriosum– Excision fibrous band

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2. Vascular sling

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Definition

• Anomalous origin of the left pulmonary artery from the posterior aspect of the right pulmonary artery.

• The anomalous left pulmonary artery courses over the right mainstem bronchus and then from right to left, posterior to the trachea or carina and anterior to the esophagus, to reach the hilum of the left lung

• Compression caused by the sling can produce obstructive emphysema and/or atelectasis of the right as well as the left lung.

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Clinical features

• Signs and symptoms– 90% severe symptoms Wheezing and stridor

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Investigation

• Chest X-ray– Anterior bowing of Right main bronchus has

deviated of lower trachea and carina to the left side.

– Left hilum is lower than normal.– Unequal aeration of both lungs +/- atelectesis

• Esophagogram– Lateral : Anterior indentation of esophagus at

carina level.

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Investigation

• Noninvasive imaging– Echocardiogram, CT scan, MRI

• Cardiac catheterization– If need confirm diagnosis.– Others congenital anomaly.

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Surgery

• Indication– If significant obstructive symptoms

• Operation– Reimplantation of the left pulmonary artery with

or without cardiopulmonary bypass.– Tracheal resection and relocation of the left

pulmonary artery.

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Thank you for your attention!!