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Truncus Arteriosus Steven H. Todman, M.D. Assistant Professor Pediatric Cardiology LSUHSC-Shreveport
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Truncus Arteriosus

Feb 23, 2016

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Truncus Arteriosus. Steven H. Todman , M.D. Assistant Professor Pediatric Cardiology LSUHSC-Shreveport. Objectives. Truncus arteriosus 1. Embryology Understand the embryologic basis of truncus arteriosus 2. Etiology, epidemiology, and genetic implications - PowerPoint PPT Presentation
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Page 1: Truncus Arteriosus

Truncus Arteriosus

Steven H. Todman, M.D.Assistant ProfessorPediatric CardiologyLSUHSC-Shreveport

Page 2: Truncus Arteriosus

ObjectivesTruncus arteriosus 1. Embryology

Understand the embryologic basis of truncus arteriosus

2. Etiology, epidemiology, and genetic implicationsUnderstand the genetic implications of truncus

arteriosus 3. Anatomy Recognize the commonly associated lesions in a

patient with truncus arteriosus Recognize the anatomic details of truncus arteriosus

Page 3: Truncus Arteriosus

Objectives4. Physiology -Understand the circulatory physiology in truncus

arteriosus 5. Natural history -Understand the natural history of a patient

with truncus arteriosus 6. Laboratory findings -Diagnose truncus arteriosus by

echocardiography, and recognize important anatomic features Diagnose truncus arteriosus by cardiac MRI/CT scan, and

recognize important anatomic features 7. Management, including complications - Plan the surgical

approach for palliation or correction of truncus arteriosus Plan appropriate preoperative medical management of a patient

with truncus arteriosus Recognize and manage early and long-term postoperative

complications following repair of truncus arteriosus

Page 4: Truncus Arteriosus

Which of the following is false?(A) Truncus arterious usually occurs as part of a

genetic syndrome.(B) Maternal diabetes is a risk factor for truncus.(C) The spiral course of the truncoaortic partition

produces the normal intertwinement of the great arteries.

(D) Deficiency or absence of the conal (infundibular) septum produces a large VSD.

(E) Deficiency of the conal septum also can contribute to deformities of the anterior tricuspid leaflet, and the medial tricuspid papillary muscle.

Page 5: Truncus Arteriosus

Which of the following is false?(A) Truncus arterious usually occurs as an

isolated cardiovascular malformation.(B) Maternal diabetes is a risk factor for truncus.(C) The spiral course of the truncoaortic partition

produces the normal intertwinement of the great arteries.

(D) Deficiency or absence of the conal (infundibular) septum produces a large VSD.

(E) Deficiency of the conal septum also can contribute to deformities of the anterior tricuspid leaflet, and the medial tricuspid papillary muscle.

Page 6: Truncus Arteriosus

Which of the following are false?(A) In type I Truncus, a short puilmonary

trunk originates from the truncus arteriousus gives rise to both pulmonary arteries.(B) When both pulmonary arteries separate from the truncus arteriosus, with no MPA, it can be type II or type III.

(C) Type A1 is the same as type I(D) Type A2 is types II and III(E) Type A4 is associated with

underdevelopment or interruption of the aortic arch.

Page 7: Truncus Arteriosus

Which of the following are false?All are true.

Page 8: Truncus Arteriosus

Which of the following are false?(A) The VSD results from absence or

deficiency of the infundibular septum.(B) The VSD is cradled between the two limbs

of septal band, and is roofed by the truncal valve cusps.

(C) Fusion of the inferior limb and the parietal band causes muscular discontinuity between the tricuspid valve and the truncal valve.

(D) The VSD in truncus arteriosus is typically membranous.

Page 9: Truncus Arteriosus

Which of the following are false?(D) The VSD in truncus arteriosus is

typically infundibular.

Page 10: Truncus Arteriosus
Page 11: Truncus Arteriosus
Page 12: Truncus Arteriosus

Which of the following are false?(A) The truncal valve is tricuspid in 69% of

cases, and bicuspid in 9% of cases.(B) The semilunar valve is in fibrous

continuity with the tricuspid valve, and usually not in fibrous continuity with the mitral valve.

(C) A right aortic arch, with mirror image branching occurs in approximately 26% of patients, second to pulmonary atresia/VSD.

(D) Interrupted aortic arch is frequently associated with DiGeorge syndrome.

Page 13: Truncus Arteriosus

Which of the following are false?(A) The truncal valve is tricuspid in 69% of

cases, and bicuspid in 9% of cases.(B) The semilunar valve is in fibrous

continuity with the mitral valve, and usually not in fibrous continuity with the tricuspid valve.

(C) A right aortic arch, with mirror image branching occurs in approximately 26% of patients, second to pulmonary atresia/VSD.

(D) Interrupted aortic arch is frequently associated with DiGeorge syndrome.

Page 14: Truncus Arteriosus

Which of the following are false?(A) Type I truncus is the most commonly observed

form, seen in about 60% of cases.(B) In truncus, the pulmonary artery that is most

frequently absent is on the side opposite of the arch, as opposed to TOF where the pulmonary artery absent is on the same side of the arch.

(C) The LAD is is frequently small and displaced leftwardly, and the conus branch is usually prominent and supplies several large branches to the RVOT.

(D) In truncus, a left coronary dominance pattern ocurs in 27% of patients, which is 3x higher than in the general population.

Page 15: Truncus Arteriosus

Which of the following are false?(A) Type I truncus is the most commonly observed

form, seen in about 60% of cases.(B) In truncus, the pulmonary artery that is

most frequently absent is on the side of the arch, as opposed to TOF where the pulmonary artery absent is on the side opposite to the arch.

(C) The LAD is is frequently small and displaced leftwardly, and the conus branch is usually prominent and supplies several large branches to the RVOT.

(D) In truncus, a left coronary dominance pattern ocurs in 27% of patients, which is 3x higher than in the general population.

Page 16: Truncus Arteriosus

Which of the following are false?(A) Abnormalities of ostial origin occur in

about 43% of cases, and high ostial origin (above the sinotubular junction) occurs often.

(B) In cases where the membranous septum is intact, and the VSD is infundibular, the AV conduction tissue is somewhat distant from the rim of the defect.

(C) Anomalies most commonly associated with truncus arteriosus are RAA, IAA, absent PDA, PDA, unilateral absence of a pulmonary artery, coronary ostial anomalies, and an incompetent truncal valve.

Page 17: Truncus Arteriosus

Which of the following are false?All are true.

Page 18: Truncus Arteriosus

Which of the following are false?(A) Extracardiac anomalies, present in 25% of

cases include skeletal deformities, hydroureter, and bowel malrotation.

(B) Hypertensive pulmonary vascular disease develops more rapidly and to a more severe extent in truncus arteriosus than in isolated VSD’s.

(C) Chronic truncal valve insufficiency can result in pulmonary venous hypertension.

Page 19: Truncus Arteriosus

Which of the following is false?(A) First heart sound is normal, and is often

followed by an ejection click.(B) A thrill is often palpable along the left sternal

border.(C) A third heart sound is often present, along with

a loud pansystolic murmur at the lower left sternal border.

(D) An apical diastolic high-pitched murmur is often heard from flow across the mitral valve.

(E) A diastolic high-pitched murmur is heard at the left sternal border from truncal valve insufficiency.

Page 20: Truncus Arteriosus

Which of the following is false?(A) First heart sound is normal, and is often

followed by an ejection click.(B) A thrill is often palpable along the left sternal

border.(C) A third heart sound is often present, along with

a loud pansystolic murmur at the lower left sternal border.

(D) An apical diastolic low-pitched murmur is often heard from flow across the mitral valve.

(E) A diastolic high-pitched murmur is heard at the left sternal border from truncal valve insufficiency.

Page 21: Truncus Arteriosus

Which of the following is false?(A) A continuous murmur is usually suggestive of

pulmonary ostial stenosis, but should make you think of PA/VSD with a PDA or collateral vessels than with truncus.

(B) EKG axis is normal, but may demonstrate RAD, and often shows BVH.

(C) Patients with increased blood flow will often have increased LV forces and LAE.

(D) Patients with TA with two PA’s and a pulmonary arteriolar resistance >8 Um2 are at higher risk.

(E) Complete repair is preferred in the first weeks of life.

Page 22: Truncus Arteriosus

All are true.

Page 23: Truncus Arteriosus

Which of the following is false?(A) Most surgeons prefer a valved conduit.(B) Primary late problem related to extracardiac

conduit operation is the need for replacement because of growth or deterioration and calcification of the conduit.

(C) Percutaneous valve therapy can be used for the failing conduit.

(D) Peak gradient over 50mm Hg in the conduit and RVSP >75 mm Hg is an indication to replace the conduit in adults.

(E) Infective endocarditis precautions are not warranted after repair of truncus.

Page 24: Truncus Arteriosus

Which of the following is false?(A) Most surgeons prefer a valved conduit.(B) Primary late problem related to extracardiac

conduit operation is the need for replacement because of growth or deterioration and calcification of the conduit.

(C) Percutaneous valve therapy can be used for the failing conduit.

(D) Peak gradient over 50mm Hg in the conduit and RVSP >75 mm Hg is an indication to replace the conduit in adults.

(E) Infective endocarditis precautions are warranted after repair of truncus.