cyanotic heart disease nidhi ravishankar role number: 1440
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Cyanotic Heart DiseaseNidhi 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
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
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
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
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
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!
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
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!
Features
1. Right Ventricular Hypertrophy
2. Left ventricular atropy
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
Truncus arteriosusFailure to divide
pulmonary trunk and aorta due to lack of aorticopulmonary septum formation
Most patients have accompanying VSD
Treatment
Surgical intervention
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
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
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.
TGA
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.