PVD = pulmonary Acyanotic Defects
vascular disease
Atrial Septal Defect Ventricular Septal Defect Patent Ductus Arteriosus Congenital Aortic Stenosis Pulmonic Stenosis Coarctation of the Aorta T
Failure of ductus Congenital stenosis of Perime
Persistent opening of Abnormal opening in the Obstruction of outflow to Discrete narrowing of the
Definition arteriosus to close within aortic valve, usually due to subvalv
interatrial septum after birth interventricular septum the pulmonary circulation aortic lumen
72 hours of birth a bicuspid valve + ov
- Ostium secundum ASD - #1 By location: - Prematurity: muscle is - Congenitally fused valve By location Endoca
- Ostium primum ASD or - Perimembranous (70%) less responsive to O2 - Most common - Juxtaductal → MOSTLY Abnorm
Classification partial AVSD - Muscular (20%) - Birth at high altitude - RV outflow tract defect - In thoracic or abdominal cephala
- Sinus venosus ASD - AV valve adjacent - Trisomies - Pulmonary artery aorta → very rarely
infundib
- Coronary sinus ASD - Just below aortic valve - Respiratory Distress obstruction
- Patent foramen ovale (>6mo) Syndrome interven
- Maternal exposure to Associ
Genetic syndromes: - Alcohol - Turner Syndrome - DiG
- Commonly: Tri 21, 18, & 13 - Rubella (1st trim) Congenital bicuspid aortic → Reduced blood flow [22
Etiology - Cri du Chat, Apert S - Phenytoin through aorta in utero
valve - Tri
TORCH infections - Prostaglandins → Ectopic muscle - Ot
- Second most common CHD - Most common CHD - Mostly isolated defect - Male (4x) > female pxs - Relatively common Most common associated - Most
- Female > male pxs - Maternal risk factors: - Female > male pxs - 20% = additional defect: - Rarely acquired cardiac abnormality → cyanos
Epidemiology
- 25% are diagnosed until - Diabetes, smoking, obesity - ↑ in premature CoA bicuspid aortic valve
adulthood - 5% → PDA-associated neonates - Male > female pxs
Uncomplicated → oxygenated Hemodynamic changes depend PDA → persistent shunt Significant narrowing of Impaired RV outflow → Aortic narrowing → 1. Subv
blood is shunted - LV to RV on: from descending aorta to valvular orifice→↑ afterload ↑RV pressure → RVH ↑impedance & ↑ afterload steno
- Shunt flow depends on: - Size of the defect left pulmonary artery. 1. ↑ LV systolic pressure - Coarctation is distal to resis
● Defect size - Pulmonary & systemic Magnitude depends on 2. LV hypertrophy Clinical course carotid & subclavian → 2. Veno
● Ventricular pressures resistances - Cross-sectional area 3. ↑jet velocity of blood → determined by degree of Normal blood flow to head throu
→ RA volume overload → RA → left to right shunt - Length of ductus aortic dilation obstruction & arms 3. Deox
& RV enlargement → When large → RV, LA, & LV - Pulmonary and - Mild: rarely progresses - Limited flow to descending syste
Pathophysiology tachyarrhythmias volume overload systemic resistances - Severe: if untreated, aorta 4. → sy
→ ↓ RV compliance → ↓shunt - Initially - Frank-Starling → left-to-right shunt: results in right heart If not corrected → cyan
- Then → chamber dilation, pulmonary, LA & LV failure - LVH Magnitu
systolic dysfunction → heart volume overload → left - Dilation of collateral blood through
failure symptoms ventricular-dilation → LHF vessels - bypass severity
- As early as 2 years → → if PVD → right heart coarctation → perfuse
pulmonary vascular disease disease → Eisenmenger's lower aorta - Spe
→ May cause Eisenmenger's cert
Most are asymptomatic Typically are asymptomatic Small → asymptomatic Depend on severity: Mild or moderate: - Severe → HF symptoms - Dyspn
- Dyspnea on exertion - 10% → large defects Medium → teens or adults Severe: HF before 2 y.o asymptomatic shortly after birth - Hyper
- Fatigue ● Heart failure: tachypnea, - fatigue, dyspnea, - Tachycardia Severe: - Cyanotic lower half sudde
- Recurrent LRTIs poor feeding, FTT, palpitations - Tachypnea - Exertional dyspnea - Less severe: may be cyano
Symptoms Most common in adults: frequent LRTI Large → Early CHF: - FTT & poor feeding - Exercise intolerance asymptomatic - Afte
- ↓ stamina ● PVD & reversed shunts → tachycardia, poor feeding, Most: mild & asymptomatic - Decompensation → - Claudication feed
- Palpitations (arrhythmias) dyspnea & cyanosis FTT, recurrent LRTI - Symptoms in adulthood RHF: abdominal Tortuous collateral arterial - Irrita
● Bacterial endocarditis can LA dilation → Afib - Fatigue, exertional fullness, pedal edema circulation → continuous hyp
Clinical develop Turbulent flow → ↑infection dyspnea, angina, syncope murmurs over chest in adults or c
Features
- RV heave: prominent - Harsh holosystolic murmur - Continuous, - Systolic ejection click - ↑↑↑ jugular a wave - Upper extremity HTN (>15 Modera
systolic impulse at lower left - "En barra" machine-like murmur at - Crescendo-descendo - Palpable RV heave mmHg gap) on lips,
sternal border - Left sternal border left subclavicular region systolic murmur → neck - Cres-des systolic - If coarctation proximal to left Severe
- Fixed & widened S2 splitting - Smallest are the loudest - If PVD → ↓murmur radiation murmur + palpable thrill subclavian → right arm BP cyanos
Physical
- Systolic murmur at P (not - Systolic thrill - Digital clubbing - Murmur is present from - Widened splitting + soft > left arm BP - Finge
prominent) - Mid-diastolic rumbling birth → doesn't depend on P2 - Weak & delayed femoral - RV h
- Mid-diastolic murmur at T - PVD: RV heave, loud P2 PVD development Moderate: ejection click pulse - Singl
vascular disease
Atrial Septal Defect Ventricular Septal Defect Patent Ductus Arteriosus Congenital Aortic Stenosis Pulmonic Stenosis Coarctation of the Aorta T
Failure of ductus Congenital stenosis of Perime
Persistent opening of Abnormal opening in the Obstruction of outflow to Discrete narrowing of the
Definition arteriosus to close within aortic valve, usually due to subvalv
interatrial septum after birth interventricular septum the pulmonary circulation aortic lumen
72 hours of birth a bicuspid valve + ov
- Ostium secundum ASD - #1 By location: - Prematurity: muscle is - Congenitally fused valve By location Endoca
- Ostium primum ASD or - Perimembranous (70%) less responsive to O2 - Most common - Juxtaductal → MOSTLY Abnorm
Classification partial AVSD - Muscular (20%) - Birth at high altitude - RV outflow tract defect - In thoracic or abdominal cephala
- Sinus venosus ASD - AV valve adjacent - Trisomies - Pulmonary artery aorta → very rarely
infundib
- Coronary sinus ASD - Just below aortic valve - Respiratory Distress obstruction
- Patent foramen ovale (>6mo) Syndrome interven
- Maternal exposure to Associ
Genetic syndromes: - Alcohol - Turner Syndrome - DiG
- Commonly: Tri 21, 18, & 13 - Rubella (1st trim) Congenital bicuspid aortic → Reduced blood flow [22
Etiology - Cri du Chat, Apert S - Phenytoin through aorta in utero
valve - Tri
TORCH infections - Prostaglandins → Ectopic muscle - Ot
- Second most common CHD - Most common CHD - Mostly isolated defect - Male (4x) > female pxs - Relatively common Most common associated - Most
- Female > male pxs - Maternal risk factors: - Female > male pxs - 20% = additional defect: - Rarely acquired cardiac abnormality → cyanos
Epidemiology
- 25% are diagnosed until - Diabetes, smoking, obesity - ↑ in premature CoA bicuspid aortic valve
adulthood - 5% → PDA-associated neonates - Male > female pxs
Uncomplicated → oxygenated Hemodynamic changes depend PDA → persistent shunt Significant narrowing of Impaired RV outflow → Aortic narrowing → 1. Subv
blood is shunted - LV to RV on: from descending aorta to valvular orifice→↑ afterload ↑RV pressure → RVH ↑impedance & ↑ afterload steno
- Shunt flow depends on: - Size of the defect left pulmonary artery. 1. ↑ LV systolic pressure - Coarctation is distal to resis
● Defect size - Pulmonary & systemic Magnitude depends on 2. LV hypertrophy Clinical course carotid & subclavian → 2. Veno
● Ventricular pressures resistances - Cross-sectional area 3. ↑jet velocity of blood → determined by degree of Normal blood flow to head throu
→ RA volume overload → RA → left to right shunt - Length of ductus aortic dilation obstruction & arms 3. Deox
& RV enlargement → When large → RV, LA, & LV - Pulmonary and - Mild: rarely progresses - Limited flow to descending syste
Pathophysiology tachyarrhythmias volume overload systemic resistances - Severe: if untreated, aorta 4. → sy
→ ↓ RV compliance → ↓shunt - Initially - Frank-Starling → left-to-right shunt: results in right heart If not corrected → cyan
- Then → chamber dilation, pulmonary, LA & LV failure - LVH Magnitu
systolic dysfunction → heart volume overload → left - Dilation of collateral blood through
failure symptoms ventricular-dilation → LHF vessels - bypass severity
- As early as 2 years → → if PVD → right heart coarctation → perfuse
pulmonary vascular disease disease → Eisenmenger's lower aorta - Spe
→ May cause Eisenmenger's cert
Most are asymptomatic Typically are asymptomatic Small → asymptomatic Depend on severity: Mild or moderate: - Severe → HF symptoms - Dyspn
- Dyspnea on exertion - 10% → large defects Medium → teens or adults Severe: HF before 2 y.o asymptomatic shortly after birth - Hyper
- Fatigue ● Heart failure: tachypnea, - fatigue, dyspnea, - Tachycardia Severe: - Cyanotic lower half sudde
- Recurrent LRTIs poor feeding, FTT, palpitations - Tachypnea - Exertional dyspnea - Less severe: may be cyano
Symptoms Most common in adults: frequent LRTI Large → Early CHF: - FTT & poor feeding - Exercise intolerance asymptomatic - Afte
- ↓ stamina ● PVD & reversed shunts → tachycardia, poor feeding, Most: mild & asymptomatic - Decompensation → - Claudication feed
- Palpitations (arrhythmias) dyspnea & cyanosis FTT, recurrent LRTI - Symptoms in adulthood RHF: abdominal Tortuous collateral arterial - Irrita
● Bacterial endocarditis can LA dilation → Afib - Fatigue, exertional fullness, pedal edema circulation → continuous hyp
Clinical develop Turbulent flow → ↑infection dyspnea, angina, syncope murmurs over chest in adults or c
Features
- RV heave: prominent - Harsh holosystolic murmur - Continuous, - Systolic ejection click - ↑↑↑ jugular a wave - Upper extremity HTN (>15 Modera
systolic impulse at lower left - "En barra" machine-like murmur at - Crescendo-descendo - Palpable RV heave mmHg gap) on lips,
sternal border - Left sternal border left subclavicular region systolic murmur → neck - Cres-des systolic - If coarctation proximal to left Severe
- Fixed & widened S2 splitting - Smallest are the loudest - If PVD → ↓murmur radiation murmur + palpable thrill subclavian → right arm BP cyanos
Physical
- Systolic murmur at P (not - Systolic thrill - Digital clubbing - Murmur is present from - Widened splitting + soft > left arm BP - Finge
prominent) - Mid-diastolic rumbling birth → doesn't depend on P2 - Weak & delayed femoral - RV h
- Mid-diastolic murmur at T - PVD: RV heave, loud P2 PVD development Moderate: ejection click pulse - Singl
