HEMITRUNCUS
ONE PULMONARY ARTERY FROM AORTA, OTHER ONE FROM
RVOT
NATURAL HISTORY
 INCREASE PBF
 SEVERE PAH
 SURVIVAL BEYOND 1 YR IS NOT POSSIBLE

 ASSOCIATED CARDIAC ANOMALIES MAY WOR...
PATHOPHYSIOLOGY
• FIRST DESCRIBED BY FRAENTZEL IN 1868
• RPA FROM AORTA MOST COMMON(X4-8 TIMES)
• AFFECTED LUNG IS PERFUSE...
MANAGEMENT

THE KEY IS EARLIEST REPAIR
• END TO END ANASTOMOSIS WITH A SYNTHETIC GRAFT
• INTERPOSITION OF A HOMOGRAFT PATCH
• AORTIC FLAP OR INTERPOSITION OF AUT...
LOOP HOLES
• PVRI IS FALLACIOUS(CAN BE RECTIFIED BY USING FLOW RATE BY
CATHETERIZATION AND MRI
• PVRI ASSESSMENT CAN BE IM...
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Hemitruncus

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A variant truncus arteriosus in which only one pulmonary artery originates from the truncal artery

Published in: Health & Medicine
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Hemitruncus

  1. 1. HEMITRUNCUS ONE PULMONARY ARTERY FROM AORTA, OTHER ONE FROM RVOT
  2. 2. NATURAL HISTORY  INCREASE PBF  SEVERE PAH  SURVIVAL BEYOND 1 YR IS NOT POSSIBLE  ASSOCIATED CARDIAC ANOMALIES MAY WORSEN THE PROGNOSIS EVEN FURTHER  THE PREVALENCE OF HEMITRUNCUS BEYOND INFANCY IS NOT KNOWN AS MAJORITY OF THE SERIES HAVE PATIENTS WHO ARE BELOW ONE YEAR OF AGE AND ONLY SPORADIC CASES ARE ENCOUNTERED IN LARGER SERIES  MOST COMMONLY ASSOCIATED WITH TOF
  3. 3. PATHOPHYSIOLOGY • FIRST DESCRIBED BY FRAENTZEL IN 1868 • RPA FROM AORTA MOST COMMON(X4-8 TIMES) • AFFECTED LUNG IS PERFUSED AT SYSTEMIC PRESSURE AND THE REMAINING LUNG IS EXPOSED TO THE ENTIRE OUTPUT OF THE RIGHT VENTRICLE • PULMONARY CIRCULATION OF BOTH LUNGS ARE EXPOSED TO PRESSURE AND VOLUME OVERLOAD IN HEMITRUNCUS • SERIOUS PULMONARY VASCULAR DISEASE AT THIRD MONTH :PULMONARY BLOOD FLOW, CIRCULATING VASOCONSTRICTOR , NEUROGENIC CROSSOVER FROM UNPROTECTED LUNG AND LEFT VENTRICULAR FAILURE
  4. 4. MANAGEMENT THE KEY IS EARLIEST REPAIR
  5. 5. • END TO END ANASTOMOSIS WITH A SYNTHETIC GRAFT • INTERPOSITION OF A HOMOGRAFT PATCH • AORTIC FLAP OR INTERPOSITION OF AUTOLOGOUS PERICARDIAL PATCH FOR INCREASING THE LENGTH OF THE ANOMALOUS PA • DIRECT ANASTOMOSIS TO AVOID CONDUIT RELATED PROBLEM
  6. 6. LOOP HOLES • PVRI IS FALLACIOUS(CAN BE RECTIFIED BY USING FLOW RATE BY CATHETERIZATION AND MRI • PVRI ASSESSMENT CAN BE IMPROVED BY USING THE FORMULA 1/R = 1/R1 + 1/R2 * • • *PRESTON RR, WILSON TE, EDITORS. CARDIOVASCULAR REGULATION. IN: LIPPINCOTT'S ILLUSTRATED REVIEWS: PHYSIOLOGY. PHILADELPHIA: LIPPINCOTT WILLIAMS AND WILKINS; 2012. P. 232-50
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