2. heart pathology; congenital disorders

Heart - PathologyHeart - Pathology

Congenital Heart Disease MC type of heart disease among children MC type= VSD Etiology and Pathogenesis

Multifactorial = majority of cases Developmental

Trisomy 21 (Down syndrome) MC known genetic causeEndocardial cushion defects (AVSD) & ASD

Di -George syndrome with TOF Environment= infection (congenital rubella) or teratogens

Congenital Heart Disease MC type of heart disease among children MC type= VSD Etiology and Pathogenesis

Multifactorial = majority of cases Developmental

Trisomy 21 (Down syndrome) MC known genetic causeEndocardial cushion defects (AVSD) & ASD

Di -George syndrome with TOF Environment= infection (congenital rubella) or teratogens

Dr. Krishna Tadepalli, MD, www.mletips.com

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Congenital Heart Disease Clinical Features

Malformations causing left-to-right shunt right-to-left shunt Obstruction

Mechanisms - left-to-right shunt

Congenital Heart Disease Clinical Features

Malformations causing left-to-right shunt right-to-left shunt Obstruction

Mechanisms - left-to-right shunt

Type of Defect Mechanism

Ventricular Septal Defect (VSD)

hole within the membranous or muscular portionsof the interventricular septum

Atrial Septal Defect (ASD) A hole from a septum secundum or septumprimum defect in the interatrial septum

Patent Ductus Arteriosus (PDA)

Ductus Arteriosus remains open (normally closesSoon after birth)


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Congenital Heart Disease Mechanisms - right-to-left shunt

Congenital Heart Disease Mechanisms - right-to-left shunt

Tetrology of Fallot Pulmonic stenosis right ventricular hypertrophy , VSD& overriding aorta

Transposition of Great Vessels

Aorta arises from the right ventricle and the pulmonic trunk from the left ventricle. A VSD, or ASD with PDA, is needed for survival

Truncus Arteriosus Incomplete separation of the aortic and pulmonary outflows


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Congenital Heart Disease Mechanisms - others

Congenital Heart Disease Mechanisms - others

Hypoplastic Left Heart Syndrome

hypoplasia or atresia of the aortic and mitral valves, with a small to absent left ventricle

Coarctation of Aorta outflow obstructive narrowing of the aortic lumen, proximal (infantile form) or distal (adult form) to the ductus

Total Anomalous Pulmonary Venous Return (TAPVR)

pulmonary veins drain into left innominate vein, coronary sinus, or some other site


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Congenital Heart Disease Left-to-right shunts

ASD, VSD, and Patent Ductus Arteriosus [PDA] Congenitally acyanotic Late cyanotic congenital heart disease or Eisenmenger syndrome

ASD MC congenital cardiac anomaly asymptomatic until adulthood Secundum ASD =90% of all ASDs Defect is due to deficient or fenestrated oval fossa Volume hypertrophy of RA & RV Pulmonary blood flow - 2 to 4 times normal Murmur at pulmonary area Complications = uncommon Normal postoperative survival

Congenital Heart Disease Left-to-right shunts

ASD, VSD, and Patent Ductus Arteriosus [PDA] Congenitally acyanotic Late cyanotic congenital heart disease or Eisenmenger syndrome

ASD MC congenital cardiac anomaly asymptomatic until adulthood Secundum ASD =90% of all ASDs Defect is due to deficient or fenestrated oval fossa Volume hypertrophy of RA & RV Pulmonary blood flow - 2 to 4 times normal Murmur at pulmonary area Complications = uncommon Normal postoperative survival


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Congenital Heart Disease

VSD Most common congenital cardiac anomaly (42%) Spontaneously close in half of patients

⅔ rd are associated with other defects (TOF) clinically = Size is most critical Morphology

Membranous VSD(90%) Swiss-cheese septum =Multiple muscular septum VSDs Right ventricular hypertrophy and pulmonary hypertension

are present from birth Rx = Surgical closure indicated at age 1 year with large defects


VSD Most common congenital cardiac anomaly (42%) Spontaneously close in half of patients

⅔ rd are associated with other defects (TOF) clinically = Size is most critical Morphology

Membranous VSD(90%) Swiss-cheese septum =Multiple muscular septum VSDs Right ventricular hypertrophy and pulmonary hypertension

are present from birth Rx = Surgical closure indicated at age 1 year with large defects


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PDA Majority are isolated defects (90%) Others (10%)-associated with VSD, COA, or PS, AS No functional difficulties at birth & childhood Continuous harsh machinery-like murmur PDA is Life-threatening or life-saving

prostaglandin E preservation of ductal patency ? Drug closes PDA

AVSD(endocardial cushion defect )

Superior & inferior endocardial cushions fail to fuse Inadequate formation of the AV valves

MC with Down syndrome


PDA Majority are isolated defects (90%) Others (10%)-associated with VSD, COA, or PS, AS No functional difficulties at birth & childhood Continuous harsh machinery-like murmur PDA is Life-threatening or life-saving

prostaglandin E preservation of ductal patency ? Drug closes PDA

AVSD(endocardial cushion defect )

Superior & inferior endocardial cushions fail to fuse Inadequate formation of the AV valves

MC with Down syndrome


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Congenital Heart DiseaseRight to left shunts

Tetralogy of Fallot - MC cyanotic congenital heart disease Transposition of the great arteries Tricuspid atresia Total anomalous pulmonary venous connection Truncus Arteriosus

Congenital Heart DiseaseRight to left shunts

Tetralogy of Fallot - MC cyanotic congenital heart disease Transposition of the great arteries Tricuspid atresia Total anomalous pulmonary venous connection Truncus Arteriosus


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http://www.emedicine.com/emerg/images/Large/261261cathtof.jpg

http://www.emedicine.com/emerg/images/Large/431597boot.jpg


Tetralogy of Fallot Tetra = 4

sub pulmonary stenosis Right ventricular hypertrophy VSD Aorta that overrides the VSD

Morphology Boot-shaped Heart (due to right ventricular hypertrophy) Aortic valve insufficiency or ASD (Pentology) Pink Tetralogy = Mild Sub-pulmonary stenosis

TGA TGA incompatible unless a shunt exists TGA + VSD (35%) = stable shunt TGA + Patent foramen ovale or PDA (65%)=unstable shunts

Tetralogy of Fallot Tetra = 4

sub pulmonary stenosis Right ventricular hypertrophy VSD Aorta that overrides the VSD

Morphology Boot-shaped Heart (due to right ventricular hypertrophy) Aortic valve insufficiency or ASD (Pentology) Pink Tetralogy = Mild Sub-pulmonary stenosis

TGA TGA incompatible unless a shunt exists TGA + VSD (35%) = stable shunt TGA + Patent foramen ovale or PDA (65%)=unstable shunts


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Heart - PathologyHeart - PathologyTruncus Arteriosus

Single great artery that receives blood from both ventricles Early systemic cyanosis Irreversible pulmonary HTN

Tricuspid Atresia High mortality in the first weeks underdevelopment (hypoplasia) of RV Congenital Cyanosis

Coarctation of Aora Males are affected twice females with Turner syndrome Two classic forms – infantile, adult half of cases = associated with bicuspid aortic valve Clinical features= rib notching, pan systolic murmur & thrill

hypertension in the upper extremities weak pulses and Hypotension in the lower extremities

Truncus Arteriosus Single great artery that receives blood from both ventricles Early systemic cyanosis Irreversible pulmonary HTN

Tricuspid Atresia High mortality in the first weeks underdevelopment (hypoplasia) of RV Congenital Cyanosis

Coarctation of Aora Males are affected twice females with Turner syndrome Two classic forms – infantile, adult half of cases = associated with bicuspid aortic valve Clinical features= rib notching, pan systolic murmur & thrill

hypertension in the upper extremities weak pulses and Hypotension in the lower extremities


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2. heart pathology; congenital disorders

Health & Medicine

Transcript of 2. heart pathology; congenital disorders