The HeartThe Heart

Premed 2Premed 2

PathophysiologyPathophysiology

Congenital Heart DiseaseCongenital Heart Disease

Mostly associated with chromosomal Mostly associated with chromosomal abnormalities.abnormalities.

Turner syndrome and Down Turner syndrome and Down syndromesyndrome

Rubella (German measles) can be a Rubella (German measles) can be a cause.cause.

Turner syndromeTurner syndrome or or Ullrich-Ullrich-Turner syndromeTurner syndrome (also known as (also known as "Gonadal dysgenesis"Gonadal dysgenesis”) encompasses ) encompasses several conditions, of which several conditions, of which monosomy X (absence of an entire X (absence of an entire sex chromosome) is most common. It sex chromosome) is most common. It is a is a chromosomal abnormality in in which all or part of one of the which all or part of one of the sex chromosomes is absent is absent (unaffected humans have 46 (unaffected humans have 46 chromosomes, of which two are sex chromosomes, of which two are sex chromosomes).chromosomes).

Down syndromeDown syndrome, or , or Down's Down's syndromesyndrome is a is a chromosomal disorder caused by the caused by the presence of all or part of an extra presence of all or part of an extra 21st chromosome. The condition is . The condition is characterized by a combination of characterized by a combination of major and minor differences in major and minor differences in structure. Often Down syndrome is structure. Often Down syndrome is associated with some impairment of associated with some impairment of cognitive ability and ability and physical growth, and a particular set of facial , and a particular set of facial characteristics. characteristics.

Congenital Heart Congenital Heart DiseasesDiseases

CYANOTICCYANOTIC Tetralogy of FallotTetralogy of Fallot Transposition of Transposition of

the Great vesselsthe Great vessels

NONCYANOTICNONCYANOTIC Aortic stenosisAortic stenosis Coarctation of the Coarctation of the

AortaAorta Patent Ductus Patent Ductus

ArteriosusArteriosus Atrial septal defectAtrial septal defect Ventricular septal Ventricular septal

defectdefect

Tetralogy of FallotTetralogy of Fallot

Tetralogy of Tetralogy of FallotFallot is a is a congenital heart defect which is which is classically classically understood to understood to involve four involve four anatomical anatomical abnormalities. It is abnormalities. It is the most common the most common cyanotic heart heart defect, defect, representing 55-representing 55-70%, and the most 70%, and the most common cause of common cause of blue baby syndrome. .

Tetralogy of FallotTetralogy of Fallot

Pulmonary stenosisPulmonary stenosis Ventricular septal defectVentricular septal defect Overriding aortaOverriding aorta Right ventricular hypertrophyRight ventricular hypertrophy

Transposition of the Transposition of the great vesselsgreat vessels

Aorta comes from Aorta comes from right ventricleright ventricle

Pulmonary artery Pulmonary artery comes from left comes from left ventricleventricle

TOGVTOGV

Survival possible Survival possible only in these casesonly in these cases

Coarctation of the AortaCoarctation of the Aorta

Narrowing of the Narrowing of the aortaaorta

Dilated collateral Dilated collateral circulation and circulation and pulmonary arteriespulmonary arteries

Patent Ductus arteriosusPatent Ductus arteriosus

Patent ductus Patent ductus arteriosusarteriosus ( (PDAPDA) ) is a is a congenital disorder in in heart wherein a wherein a neonate's neonate's ductus arteriosus fails to close after fails to close after birth. Symptoms . Symptoms are uncommon but are uncommon but in the first year of in the first year of life include life include increased work of increased work of breathing and poor breathing and poor weight gain. weight gain.

With age, the With age, the PDA may lead to PDA may lead to congestive congestive heart failure if if left uncorrected.left uncorrected.

Ventricular septal defectVentricular septal defect

Opening between Opening between the right and left the right and left ventricleventricle

Symptoms depend Symptoms depend on the sizeon the size

Large defects: Large defects: pulmonary pulmonary hypertension and hypertension and eventually, right –eventually, right –sided heart failuresided heart failure

Atrial septal defectAtrial septal defect

Septum primum: Septum primum: affects the lower affects the lower part of the septum; part of the septum; near the AV valvesnear the AV valves

ASDASD

Septum secundum: Septum secundum: defect in the fossa defect in the fossa ovalisovalis

Sinus venosus: Sinus venosus: affects the upper affects the upper part of the septum; part of the septum; near the SVCnear the SVC

ASDASD

Most of the signs and symptoms Most of the signs and symptoms appear at adulthoodappear at adulthood

Pulmonary hypertensionPulmonary hypertension

Rheumatic FeverRheumatic Fever Affects children 5 to 15 years oldAffects children 5 to 15 years old Occurs after sore throat: Streptococcus Occurs after sore throat: Streptococcus

pyogenespyogenes Damage secondary to antibodiesDamage secondary to antibodies Multisystemic:Multisystemic:

joint painsjoint painsfever, malaisefever, malaisesubcutaneous nodules, erythema subcutaneous nodules, erythema marginatummarginatumSydenham chorea:involuntary movements, Sydenham chorea:involuntary movements, bizarre grimaces, emotionally unstablebizarre grimaces, emotionally unstable

RFRF

Endocarditis Endocarditis causes causes inflammation and inflammation and fibrosis of the fibrosis of the valvesvalves

Most affected: Most affected: MITRAL VALVEMITRAL VALVE

““fish- mouth fish- mouth deformity”deformity”

Mitral stenosis: Mitral stenosis: pressure is higher pressure is higher in the left atriumin the left atrium

Infective endocarditisInfective endocarditis

Infective endocarditisInfective endocarditis is a form of is a form of endocarditis caused by infectious caused by infectious agents. agents.

Vegetations on the valves: fibrin + Vegetations on the valves: fibrin + inflammatory cells + bacteriainflammatory cells + bacteria

Acute endocarditis:Staphylococcus Acute endocarditis:Staphylococcus aureusaureus

Subacute endocarditis: abnormal Subacute endocarditis: abnormal valves; Streptococcus viridansvalves; Streptococcus viridans

Most commonly involved: MITRAL Most commonly involved: MITRAL VALVEVALVE

IV drug users, staphylococcal cause: IV drug users, staphylococcal cause: TRICUSPID VALVETRICUSPID VALVE

Acute/ Subacute endocarditis

Marantic endocarditis

Other forms of Other forms of endocarditisendocarditis

Marantic endocarditisMarantic endocarditis Nonbacterial thrombotic Nonbacterial thrombotic

endocarditisendocarditis Seen in cancer and debilitated Seen in cancer and debilitated

patientspatients Sterile vegetations, emboliSterile vegetations, emboli

Diseases of the valvesDiseases of the valves

Often due to Often due to rheumatic feverrheumatic fever

Mitral valve Mitral valve prolapseprolapse

““floppy valve” or floppy valve” or parachute parachute deformitydeformity

Ischemic Heart diseaseIschemic Heart disease

Narrowing of the Narrowing of the coronary arteriescoronary arteries

Common in :Common in :

obesityobesity

sedentary lifestylesedentary lifestyle

high cholesterolhigh cholesterol

hypertensionhypertension

diabetesdiabetes

Angina pectorisAngina pectoris

Episodes of chest pain due to Episodes of chest pain due to inadequate oxygenation of the inadequate oxygenation of the myocardiummyocardium

Stable angina: most common; pain Stable angina: most common; pain on exertion, relieved by rest or on exertion, relieved by rest or medications;medications;

narrowing of the coronary vesselsnarrowing of the coronary vessels

Unstable angina: prolonged or Unstable angina: prolonged or recurrent pain AT REST; recurrent pain AT REST; atherosclerotic plaque and atherosclerotic plaque and thrombosis; may indicate impending thrombosis; may indicate impending heart attackheart attack

Prizmetal angina: intermittent chest Prizmetal angina: intermittent chest pain at rest; vasospasmpain at rest; vasospasm

Myocardial InfarctionMyocardial Infarction

Coronary artery occlusionCoronary artery occlusion Coagulative necrosisCoagulative necrosis Cells: neutrophils, macrophages, Cells: neutrophils, macrophages,

fibroblastsfibroblasts Increase of cardiac enzymes: CK-Increase of cardiac enzymes: CK-

MB, Troponin I and LDHMB, Troponin I and LDH

MI ComplicationsMI Complications

Arrythmias: most common cause of Arrythmias: most common cause of death in the 1death in the 1stst 6 hours 6 hours

Myocardial rupture: most common Myocardial rupture: most common cause of death with 4cause of death with 4thth to 7 to 7thth day day

Ventricular aneurysm: within 4 to 6 Ventricular aneurysm: within 4 to 6 monthsmonths

Congestive Heart FailureCongestive Heart Failure

Left-sidedLeft-sided Pulmonary Pulmonary

congestion: congestion: dyspnea, dyspnea, orthopneaorthopnea

Pleural effusion, Pleural effusion, hydrothoraxhydrothorax

Right-sidedRight-sided Enlarged, Enlarged,

congested liver congested liver and spleenand spleen

Distended neck Distended neck veinsveins

Renal Renal hypoxiahypoxiaperipherperipheral edemaal edema

Congestive Heart failureCongestive Heart failure

Left sidedLeft sided MIMI HypertensionHypertension Aortic and mitral Aortic and mitral

valve diseasevalve disease Myocardial Myocardial

diseasesdiseases

Right sidedRight sided Left-sided heart Left-sided heart

failure(most failure(most common)common)

Pulmonary Pulmonary hypertensionhypertension

Tricuspid and Tricuspid and pulmonary valve pulmonary valve diseasedisease

Tumors of the HeartTumors of the Heart

Myxoma of the left Myxoma of the left atrium: most atrium: most common cardiac common cardiac tumor; adultstumor; adults

A A rhabdomyomarhabdomyoma is a is a benign tumor of of striated muscle. . Cardiac Cardiac rhabdomyomas are rhabdomyomas are the most common the most common primary tumor of primary tumor of the heart in infants the heart in infants and children.and children.

It is most It is most commonly commonly associated with the associated with the tongue[2] and and heart,,[3] but can also but can also occur in other occur in other locations.locations.

The The malignant form of form of rhabdomyoma is rhabdomyoma is called called rhabdomyosarcoma..

Metastatic tumors Metastatic tumors are more common are more common than primary than primary tumorstumors