sutton 7 transposition of the great arteries

Oleh : Reni Indrastuti

Pembimbing : dr. H. Edy Moeljono, Sp.Rad (K) RA

Oleh : Reni Indrastuti

Pembimbing : dr. H. Edy Moeljono, Sp.Rad (K) RA

Text Book Reading David Sutton Radiology and Imaging 7thth ed vol.1 Section 2, page 385-389

CONGENITAL HEART DISEASE

• VSD• ASD• PDA• PS• Coarctation of aorta• Aortic stenosis• TOF• TGATGA• AV septal defect• Pulmonary atresia• Single ventricle• Tricuspid atresia

• Mitral valve abnormalities• Hypoplastic left heart syndrome• PAPVC• TAPVC• Truncus arteriosus• Ebstein anomaly• Sinus of Valsava Fistula• Double outlet ventricle• Great arterial anomaly• Coronary anomaly• Arteriovenous malformation• Systemic venous Anomaly

Congenital Heart DiseasesCongenital Heart DiseasesCongenital Heart DiseasesCongenital Heart Diseases

David Sutton Radiology and Imaging

Cyanotic Heart DiseasesCyanotic Heart DiseasesCyanotic Heart DiseasesCyanotic Heart Diseases

Learningradiology.com

• Congenital Heart Diseases

Acyanotic : VSD, ASD, PDA, ECD, PAPVR Pulmonary Stenosis

Cyanotic : TAPVR, TAP, TGA, TOF, Pulmonary atresia, Tricuspidal atresia, Ebstein Anomaly

OVERVIEWOVERVIEWOVERVIEWOVERVIEW

• transposition of the great arteries (TGA) is a serious heart defect which the two main arteries leaving the heart are reversed (transposed). The hallmark of TGA is ventriculoarterial discordance, which aorta arises from RV, pulmonary artery arises from LV

www.mayoclinic.com/health/transpositio-the-great-arteries, www.medscape.com

DEFINITION

• 1 of 4000-5000 biths, annual incidence 20-30 per 100.000 live birth• history of diabetic mother

• second most common cause of cyanosis in infancy (5-7% of congenital heart disease)

• No race predilection• 60-70% male predominance

Epidemiology

Unknown risk factorsCause

OVERVIEWOVERVIEWOVERVIEWOVERVIEW

About 1/3 have VSD About ¼ to ½ have patent ductus

Some have ASD Pulmonary stenosis

Associated abnormality

www.cdc.govwww.learningradiology.com

The mother having : viral illness during pregnancy

poor nutrition during pregnancy excessive amount of alkohol during pregnancy

older than 40 y.o Diabetic

The baby having : down syndrome

Risk Factors

CLINICAL MANIFESTATIONCLINICAL MANIFESTATIONCLINICAL MANIFESTATIONCLINICAL MANIFESTATION

Blueness of the skin Shortness of breath

Poor feeding

Symptoms

Tachypneu, tachycardi No murmur unless other lession present

palpable right ventriculer pulse since RV faces systemic pressure

accentuated S2 due to aortic valve closure located anterior under chest wall

Physical Examination

www.cdc.gov

ANATOMYANATOMYANATOMYANATOMY

• Pulmonic valve is• Anterior• Lateral• Superior

To aortic valve

Normal Relationship of Pulmonary to Aortic Valve

PALS


ANATOMYANATOMYANATOMYANATOMY

In transposition, pulmonic valve is


• RV : trabeculated, with pulmonic infundibulum

• LV : smooth

TYPESTYPESTYPESTYPES


D-trans

David Sutton Radiology and Imaging

L-trans

Aorta is anterior and right to pulmonary artery

Aorta is anterior and left to pulmonary artery

IMAGING MODALITYIMAGING MODALITYIMAGING MODALITYIMAGING MODALITY

CHEST X RAYCHEST X RAYCHEST X RAYCHEST X RAY

RSNA 2007; 27:1323–1334

narrowing of the superior mediastinum

enlargement of the cardiac silhouette with abnormal convexity of the right atrial border

increased vascular flow — typical features of transposition of the great arteries

characteristic cardiomediastinal silhouette: the egg-on-a-string signthe egg-on-a-string sign

TREATMENTTREATMENTTREATMENTTREATMENT

• Medical:– PGE1– O2 (3L/minute)– Correct :

• acidosis ,hypoglycemia. • electrolyte disturbances.

• Transcatheter :– BAS

• Surgical:– Arterial switch (Jatene operation) at 7-15 days– Atrial switch ( Senning operation) at 6-9 months

Awni Al- Madani., MD,Congenital Heart Disease

COMPLICATIONCOMPLICATIONCOMPLICATIONCOMPLICATION

Lack of oxygen to the tissues

Heart Failure Lung damage

Complication of TGA

• Narrowing coronary arteries• Arhytmia

• Heart muscle weakness or stiffness leading to heart failure

• leaky heart valves

Complication of surgery

www.mayoclinic.com/health/transpositio-the-great-arteries,

DDDDDDDD

RESUMERESUMERESUMERESUME

QUIZQUIZQUIZQUIZ

Male infant in first day of life transferred from outside hospital with low O2 saturation. History of diabetic mother (+). CXR is showed beside.1.Please describe this picture2.What is the most likely diagnosis?

HIDDEN slide

Infant of diabetic mother

• Congenital heart defects occur in up to 8.5 per 100 lives births of infants of diabetic mothers.21 The congenital heart defects identified in offspring of diabetic mothers include double‐outlet right ventricle, truncus arteriosus, transposition of the great arteries, ventricular septal defect, and hypoplastic left heart syndrom

• High maternal haemoglobin A1c values during early pregnancy are associated with increased risk of malformations

• Hyperglycaemia has a direct influence on the proliferation and migration of neural crest cells which are critical in the development of the heart and brain

• Maternal diabetes is associated with induction of placental genes associated with chronic stress and inflammation,28 and recent investigations have even implicated a potential role for inflammation in the evolution of maternal diabetes‐induced embryopathy

• the finding that altered fetal heart function has been observed as early as 12–14 weeks10,30 and is more prevalent where there is worse metabolic control10 could suggest a haemodynamic influence, even in the first trimester, which may contribute further to the evolution of structural pathology.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1861084/?report=classic

PGE1

• The medication of prostaglandin E1 (alprostadil) helps keep connection between the aorta and pulmonary artery open (ductus arteriosus), increasing blood flow and improving mixing of oxygen poor and oxygen rich blood until surgey can be performed


Complication of TGA

• Baby’s tissues will receive too litle oxygen (hypoxia). Unless there’s some mixing oxygen poor blood and oxygen rich blood within baby’s body.

• Heart failure, a condition when heart can’t pump enough blood to meet the body’s needs-may develop over time because the right ventricle is pumping under higher pressure than usual. This added stress may make the muscle of the right venricle stiff or weak

• The lack of oxygenated blood causes the damage of the lungs, making breathing difficult


PATHOPATHOEMBRYOEMBRYOLOGYLOGYPATHOPATHOEMBRYOEMBRYOLOGYLOGY

Abnormal looping of the great vessels is believed to occur. In the normal heart a bilateral conus develop beneath both semilunar valves with progressive development of the subpulmonary conus and regression of the subaortic conus, resulting in the great vessels arising from appropriate ventricle. In D-trans the reverse occurs with progressive growth of the subaortic conus and regression of the subaortic conus resulting in transposition of the great arteries

Bcm

.edu

Because of the looping, the septating arterial trunk will be dragged to theright , and twisted as well.As a result the ascending aortacomes to lie to the right of thepulmonary artery.Note that the looping brings the trunk close to the AV canal.

The aorta is now poorly placed to attach itself to the left ventricle and some mechanism is needed todrag it to the left but still leave the PA over the rightventricle. (One might wonder why the truncal septum does not seal off anteriorly above the sixth aortic arches, and so make the anterior channel the pulmonary artery.)

RADIOLOGICAL FEATURES • CXR may exclude non cardiac causes of cyanosis e.g. RDS. .

Meconium aspiration, Diaphgramatic hernia, PneumothoraxPulmonary Vascular Markings

Decreased Increased

Heart Size Heart Size

Normal Increased Increased( “Boot shaped”) (“ Wall-to-Wall”) TOF Ebstein (“ egg-on-end”) D-TGA Aortic Arch \ Mediastinum

Abdominal Situs

TABLE 51.11 Pulmonary Vascular Patterns

• Increased vascularity (active) without cyanosis Atrial septal defect Ventricular septal defect Patent ductus arteriosus Aortic-pulmonary window Ruptured aneurysm of sinus of Valsalva Coronary artery fistula Partial anomalous pulmonary venous returnIncreased vascularity (active) with cyanosis Total anomalous pulmonary venous return (types 1, 2) Persistent truncus arteriosus Complete endocardial cushion defect

• Transposition of the great vessels complex Single ventricle (without pulmonary stenosis)Increased vascularity (passive) Total anomalous pulmonary venous return (type 3) Pulmonary vein atresia Hypoplastic left heart syndrome (in failure) Cor triatriatumDecreased vascularity Tetralogy of Fallot Pseudotruncus arteriosus Hypoplastic right heart syndrome (right-to-left shunt) Tricuspid atresia Pulmonary atresia Tricuspid stenosis Hypoplastic RV Ebstein anomaly Uhl anomaly Trilogy of Fallot Single ventricle or transposition of great vessels with pulmonary stenosis or atresia Tricuspid or pulmonary insufficiency with right-to-left shuntNormal vascularity Left heart lesions Coarctation of the aorta Interrupted aortic arch Hypoplastic left heart syndrome (before failure develops) Endocardial fibroelastosis Cardiomyopathy Aberrant left coronary artery Mitral stenosis and insufficiency Aortic stenosis and insufficiency Cor triatriatum Right heart lesions (without right-to-left shunt) Pulmonary stenosis or insufficiency Tricuspid insufficiency

sutton 7 transposition of the great arteries

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