The Heart Premed 2 Pathophysiology

Congenital Heart Disease Mostly associated with chromosomal abnormalities. Mostly associated with chromosomal abnormalities. Turner syndrome and Down syndrome Turner syndrome and Down syndrome Rubella (German measles) can be a cause. Rubella (German measles) can be a cause.

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

Down syndrome, or Down's syndrome is a chromosomal disorder caused by the presence of all or part of an extra 21st chromosome. The condition is characterized by a combination of major and minor differences in structure. Often Down syndrome is associated with some impairment of cognitive ability and physical growth, and a particular set of facial characteristics. Down syndrome, or Down's syndrome is a chromosomal disorder caused by the presence of all or part of an extra 21st chromosome. The condition is characterized by a combination of major and minor differences in structure. Often Down syndrome is associated with some impairment of cognitive ability and physical growth, and a particular set of facial characteristics. chromosomal disorder21st chromosomecognitivephysical growth chromosomal disorder21st chromosomecognitivephysical growth

Congenital Heart Diseases CYANOTIC CYANOTIC Tetralogy of Fallot Tetralogy of Fallot Transposition of the Great vessels Transposition of the Great vessels NONCYANOTIC NONCYANOTIC Aortic stenosis Aortic stenosis Coarctation of the Aorta Coarctation of the Aorta Patent Ductus Arteriosus Patent Ductus Arteriosus Atrial septal defect Atrial septal defect Ventricular septal defect Ventricular septal defect

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

Tetralogy of Fallot Pulmonary stenosis Pulmonary stenosis Ventricular septal defect Ventricular septal defect Overriding aorta Overriding aorta Right ventricular hypertrophy Right ventricular hypertrophy

Transposition of the great vessels Aorta comes from right ventricle Aorta comes from right ventricle Pulmonary artery comes from left ventricle Pulmonary artery comes from left ventricle

TOGV Survival possible only in these cases Survival possible only in these cases

Coarctation of the Aorta Narrowing of the aorta Narrowing of the aorta Dilated collateral circulation and pulmonary arteries Dilated collateral circulation and pulmonary arteries

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

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

Ventricular septal defect Opening between the right and left ventricle Opening between the right and left ventricle Symptoms depend on the size Symptoms depend on the size Large defects: pulmonary hypertension and eventually, right –sided heart failure Large defects: pulmonary hypertension and eventually, right –sided heart failure

Atrial septal defect Septum primum: affects the lower part of the septum; near the AV valves Septum primum: affects the lower part of the septum; near the AV valves

ASD Septum secundum: defect in the fossa ovalis Septum secundum: defect in the fossa ovalis Sinus venosus: affects the upper part of the septum; near the SVC Sinus venosus: affects the upper part of the septum; near the SVC

ASD Most of the signs and symptoms appear at adulthood Most of the signs and symptoms appear at adulthood Pulmonary hypertension Pulmonary hypertension

Rheumatic Fever Affects children 5 to 15 years old Affects children 5 to 15 years old Occurs after sore throat: Streptococcus pyogenes Occurs after sore throat: Streptococcus pyogenes Damage secondary to antibodies Damage secondary to antibodies Multisystemic: Multisystemic: joint pains fever, malaise subcutaneous nodules, erythema marginatum Sydenham chorea:involuntary movements, bizarre grimaces, emotionally unstable

RF Endocarditis causes inflammation and fibrosis of the valves Endocarditis causes inflammation and fibrosis of the valves Most affected: MITRAL VALVE Most affected: MITRAL VALVE “fish- mouth deformity” “fish- mouth deformity” Mitral stenosis: pressure is higher in the left atrium Mitral stenosis: pressure is higher in the left atrium

Infective endocarditis Infective endocarditis is a form of endocarditis caused by infectious agents. Infective endocarditis is a form of endocarditis caused by infectious agents. endocarditis Vegetations on the valves: fibrin + inflammatory cells + bacteria Vegetations on the valves: fibrin + inflammatory cells + bacteria Acute endocarditis:Staphylococcus aureus Acute endocarditis:Staphylococcus aureus Subacute endocarditis: abnormal valves; Streptococcus viridans Subacute endocarditis: abnormal valves; Streptococcus viridans Most commonly involved: MITRAL VALVE Most commonly involved: MITRAL VALVE IV drug users, staphylococcal cause: TRICUSPID VALVE IV drug users, staphylococcal cause: TRICUSPID VALVE

Acute/ Subacute endocarditis Marantic endocarditis

Other forms of endocarditis Marantic endocarditis Marantic endocarditis Nonbacterial thrombotic endocarditis Nonbacterial thrombotic endocarditis Seen in cancer and debilitated patients Seen in cancer and debilitated patients Sterile vegetations, emboli Sterile vegetations, emboli

Diseases of the valves Often due to rheumatic fever Often due to rheumatic fever Mitral valve prolapse Mitral valve prolapse “floppy valve” or parachute deformity “floppy valve” or parachute deformity

Ischemic Heart disease Narrowing of the coronary arteries Narrowing of the coronary arteries Common in : Common in :obesity sedentary lifestyle high cholesterol hypertensiondiabetes

Angina pectoris Episodes of chest pain due to inadequate oxygenation of the myocardium Episodes of chest pain due to inadequate oxygenation of the myocardium Stable angina: most common; pain on exertion, relieved by rest or medications; Stable angina: most common; pain on exertion, relieved by rest or medications; narrowing of the coronary vessels narrowing of the coronary vessels

Unstable angina: prolonged or recurrent pain AT REST; atherosclerotic plaque and thrombosis; may indicate impending heart attack Unstable angina: prolonged or recurrent pain AT REST; atherosclerotic plaque and thrombosis; may indicate impending heart attack Prizmetal angina: intermittent chest pain at rest; vasospasm Prizmetal angina: intermittent chest pain at rest; vasospasm

Myocardial Infarction Coronary artery occlusion Coronary artery occlusion Coagulative necrosis Coagulative necrosis Cells: neutrophils, macrophages, fibroblasts Cells: neutrophils, macrophages, fibroblasts Increase of cardiac enzymes: CK-MB, Troponin I and LDH Increase of cardiac enzymes: CK-MB, Troponin I and LDH

MI Complications Arrythmias: most common cause of death in the 1 st 6 hours Arrythmias: most common cause of death in the 1 st 6 hours Myocardial rupture: most common cause of death with 4 th to 7 th day Myocardial rupture: most common cause of death with 4 th to 7 th day Ventricular aneurysm: within 4 to 6 months Ventricular aneurysm: within 4 to 6 months

Congestive Heart Failure Left-sided Left-sided Pulmonary congestion: dyspnea, orthopnea Pulmonary congestion: dyspnea, orthopnea Pleural effusion, hydrothorax Pleural effusion, hydrothorax Right-sided Right-sided Enlarged, congested liver and spleen Enlarged, congested liver and spleen Distended neck veins Distended neck veins Renal hypoxia  peripheral edema Renal hypoxia  peripheral edema

Congestive Heart failure Left sided Left sided MI MI Hypertension Hypertension Aortic and mitral valve disease Aortic and mitral valve disease Myocardial diseases Myocardial diseases Right sided Right sided Left-sided heart failure(most common) Left-sided heart failure(most common) Pulmonary hypertension Pulmonary hypertension Tricuspid and pulmonary valve disease Tricuspid and pulmonary valve disease

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

A rhabdomyoma is a benign tumor of striated muscle. Cardiac rhabdomyomas are the most common primary tumor of the heart in infants and children. A rhabdomyoma is a benign tumor of striated muscle. Cardiac rhabdomyomas are the most common primary tumor of the heart in infants and children. benigntumorstriated muscle benigntumorstriated muscle It is most commonly associated with the tongue [2] and heart, [3] but can also occur in other locations. It is most commonly associated with the tongue [2] and heart, [3] but can also occur in other locations. tongue [2]heart [3] tongue [2]heart [3] The malignant form of rhabdomyoma is called rhabdomyosarcoma. The malignant form of rhabdomyoma is called rhabdomyosarcoma.malignant rhabdomyosarcomamalignant rhabdomyosarcoma

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