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Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

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Page 1: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Cyanotic Heart DiseaseNidhi Ravishankar

Role number: 1440

Page 2: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Introduction – what is cyanosis?

Bluish discolouration of the skin and mucous membranes caused by accumulation of deoxygenated hemoglobin in the affected area.

Peripheral and central cyanosis

Peripheral: secondary to low cardiac output leading to bluing of the periphery (toes, nails, nose); normal PaO2

Central: bluish discolouration of the skin and mucous membranes with deoxygenated hemoglobin > 3g/dL in arterial blood and > 5g/dL in capillary blood; low PaO2 and SaO2

Page 3: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Cyanotic heart disease A group of heart defects present at birth resulting in a low blood oxygen level

increased pulmonary vascularity total anomalous pulmonary venous return (TAPVR) (types I and II) transposition of the great arteries (TGA) truncus arteriosus (types I, II and III) large AVSD single ventricle without pulmonary stenosis

decreased pulmonary vascularity tetralogy of Fallot  pentalogy of Cantrell many other combined and infrequent anomalies such as

double outlet right ventricle (DORV) with pulmonary stenosis single ventricle with pulmonary stenosis Ebstein anomaly with atrial septal defect Uhl anomaly

Page 4: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Right-to-left shunt (5 Ts)

Right-to-Left shunts: eaRLy cyanosis.

Tetralogy of FallotTransposition of the great arteries Tricuspid atresiaTruncus arteriosus Total anomalous pulmonary

venous return

Page 5: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Tetralogy of FallotMost common cause of

cyanotic congenital heart disease and accounts for 5% of all congenital cardiac malformations

Anterolateral displacement of the infundibular septum leading to abnormal septation between the pulmonary trunk and aortic root

Page 6: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Four features - PROVe

1. Pulmonary infundibular stenosis (most important determinant for prognosis)

2. Right ventricular hypertrophy (RVH)— boot-shaped heart on CT

3. Overriding aorta

4. VSD

Page 7: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Clinical manifestation Severity depends on the

level of pulmonary outflow obstruction

Mild: Resembles an isolated

VSD due to left to right sided pressure gradient

Severe: Early cyanosis As child’s heart grows,

there is progressive weakening

Infective endocarditis

Paradoxical and systemic embolization

Right ventricular failure is RARE!

Page 8: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Clinical signs

1. Polycythemia

2. Hypertrophic osteoarthropathy

3. Harsh SEM over pulmonic area and left sternal border; single S2

Treatment: surgical correction

Squatting: increases SVR, decreases right-to-left shunt, less cyanosis

Page 9: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Transposition of Great Arteries/Vessels

Discordant connection of the ventricles to their vesicular outflow

Abnormal formation of the truncal and aortopulmonary septa so that the aorta arises from the right ventricle and the pulmonary artery emanates from the left ventricle

Incompatible with postnatal life unless shunt such as VSD, PDA or patent foramen ovale is placed!

Page 10: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Features

1. Right Ventricular Hypertrophy

2. Left ventricular atropy

Page 11: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Clinical manifestations

Cyanosis: Prognosis depending on the magnitude of shunting, the degree of tissue hypoxia, and the ability of the right ventricle to maintain systemic pressures.

No murmur auscultated, but loud single S2

Treatment:

Surgical interventionWithout surgery, even with stable shunting, patients die

within first few months of lifeYoutube link: https://www.youtube.com/watch?

v=lgBZI_3ltTc

Page 12: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Truncus arteriosusFailure to divide

pulmonary trunk and aorta due to lack of aorticopulmonary septum formation

Most patients have accompanying VSD

Treatment

Surgical intervention

Page 13: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Total anomalous pulmonary venous return (TAPVR)

Pulmonary veins drain out into right heart circulation (eg. SVC, coronary sinus, etc)

Associated with ASD and sometimes PDA to allow for right-to-left shunting to maintain CO.

Treatment

Surgical intervention

Page 14: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Important notes:

Signs: cyanosis, clubbed fingers, abnormal heart sounds

Tests: chest X-rays, arterial blood gas test

Treatment: surgical interventions

TOF: PROVe and boot-shaped CT, single S2, harsh 3/6 systolic ejection murmur; TGA: opposite

Page 15: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

Tetralogy of fallot

A 1-day-old infant in the general care nursery born at full term by uncomplicated spontaneous vaginal delivery is noted to have cyanosis of the oral mucosa. The baby otherwise appears comfortable. On exam, respiratory rate is 40 and pulse oximetry is 80%. A right ventricular lift is palpated, S1 is normal, S2 is single, and a harsh 3/6 systolic ejection murmur is heard at the left upper sternal border.

Page 16: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

TGA

Page 17: Cyanotic Heart Disease Nidhi Ravishankar Role number: 1440

References

Kumar, Vinay, Abul K. Abbas, Nelson Fausto, Stanley L. Robbins, and Ramzi S. Cotran. Robbins and Cotran Pathologic Basis of Disease. Philadelphia: Elsevier Saunders, 2014. Print.

Le, Tao., Bhushan, Vikas., Sochat, Matthew. First Aid for the Usmle Step 1 2015. United States: McGraw Hill Education, 2015. Print.