Examination and Investigations of CYANOTIC CONGENITAL HEART DISEASE

      Collateral circulation / anastomoses in coarcta­tion of aorta       Examination of precordium :

  • A systolic thrill may be felt in the suprasternal notch.
  • There is a heaving left ventricular apical impulse due to pressure over­load of LV.
  • A systolic murmur may be heard at the right upper
  • Prominent pulsations may be felt in the intercostal areas.
  • Sl and S2 are normal.
  • ernal border and between the scapulae.
Clinical features Coarctation of the Aorta 1

Examination and Investigations of CYANOTIC CONGENITAL HEART DISEASE

coarcta­tion of aorta Physical Exam

  • Cyanosis, rarely
  • In infancy, also may have heart failure, failure to thrive, irritability, tachypnea, and dyspnea
  • HTN
  • Pulse disparity: Pulse delay in femoral vs radial artery and increased amplitude in brachial vs femoral pulse
  • Prominent neck pulsations
  • Epistaxis
  • Delayed, weak, or absent lower extremity pulse
  • Prominent left ventricular impulse
  • 10 mmHg compared with lower extremity.
  • Bruit (coarctation, collaterals, patent ductus arteriosus)
  • Murmur (aortic stenosis or insufficiency, ventricular septal defect, rarely mitral valve)
  • S4systolic ejection click
  • BP: Evaluation shows upper limb systemic HTN and a differential
  • Funduscopy: Corkscrew tortuosity of retinal arterioles
  • Extensive collaterals develop from branches of the subclavian, internal mammary, superior intercostal, and axillary arteries.
  • Left arm diameter may be smaller than the right.

Collateral circulation / anastomoses in coarcta­tion of aorta

  • · Internal mammary artery and deep inferior epi­gastric artery, branch of external iliac artery.
  • · Lateral thoracic branch of axillary artery with
  • · The transverse cervical artery, a branch of sub­clavian artery communicates with posterior in­tercostal arteries.
  • · Anterior intercostal branches of internal mam­mary artery and posterior intercostals, branches of descending aorta.
  • posterior intercostal arteries.
  • There is notching of the ribs seen on X-ray due to these anastomoses, in the 3rd to 9th ribs.
  • The 10th to 12th aortic intercostal arteries do not anastomose with internal mammary arteries so notch­ing is absent in 10th to 12th rib.
  • The first 2 or 3 intercostal spaces are supplied by supreme intercostal artery posteriorly which is a branch of subclavian artery. Therefore, there is no notching of the upper three ribs.

Complications of coarcta­tion of aorta

  • · Left ventricular failure
  • · Infective endocarditis
  • · Aortic rupture
  • · Dissecting aneurysm

Investigations Findings —

  • Doppler exam of pulses reveals disparity.
  • Transesophageal echocardiography
  • Echocardiography for coexisting cardiac anomalies
  • MRA/MRI

Xray:  

  • dilated left subclavian artery may be seen as a prominence in the left superior mediastinum.
  • CXR may show rib notching, “3” sign, rarely cardiomegaly.
  • A barium study of esophagus shows reverse 3 sign which is due to indentations on the left side of the barium-filled oesophagus. The upper one is caused by pre-stenotic dilatation of aorta and lower one by post-stenotic dilatation of aorta.
  • There is notching of the ribs seen on X-ray due to anastomoses, in the 3rd to 9th ribs, on the lower borders.

ECG

  • ECG may show right ventricular hypertrophy in neonates but left ventricular hypertrophy in older patients.
  • Shows left ventricular hypertrophy, Left axis devia­tion (LAD), left bundle branch block, sometimes right ventricular hypertrophy.

2D echocardiogram

  • Shows the interrupted arch, LVH, dilated ascending aorta and arch.

Doppler study

  • Localises the site of the coarct and measures the gra­dient.
Localises the site of the coarct and measures the gra­dient.

Treatment

  • Surgical repair is done at age 5-20 years. Synthetic vascular graft may be needed when narrowed aortic segment is long or there is an­eurysm.

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