CONGENITAL SUMMARY

Left atrium to right atrium, left-to-right

What is the core shunt direction in Atrial Septal Defect

Right atrium and right ventricle volume overload and increased pulmonary blood flow

What are the main hemodynamic effects of Atrial Septal Defect

Usually asymptomatic

What is the classic clinical presentation of a small to moderate Atrial Septal Defect

Exercise intolerance, dyspnea, and failure to thrive

What are three symptoms seen in a large Atrial Septal Defect

Wide fixed split S2 and systolic ejection murmur

What are the characteristic heart sounds and murmur of Atrial Septal Defect

Left upper parasternal border

Where is the systolic ejection murmur of Atrial Septal Defect loudest

Right atrial enlargement, right ventricular hypertrophy, right axis deviation, and right bundle branch block

What are four ECG findings associated with Atrial Septal Defect

Increased pulmonary vascular markings, dilated main pulmonary artery, right atrium and right ventricle enlargement, and jug-handle appearance

What are four chest X-ray findings associated with Atrial Septal Defect

Secundum

Which type is the most common Atrial Septal Defect

Holt-Oram

Which genetic syndrome is associated with Atrial Septal Defect

Pulmonary hypertension leading to Eisenmenger syndrome and atrial arrhythmias

What are two natural history complications of Atrial Septal Defect

Device closure

What is the preferred management for secundum Atrial Septal Defect

Surgery

What treatment is indicated for secundum Atrial Septal Defect if it is unsuitable for device closure

Wide, fixed split S2

What is the classic clinical exam clue for Atrial Septal Defect

Left ventricle to right ventricle, left-to-right during systole

What is the core shunt direction and timing in Ventricular Septal Defect

Left ventricular volume overload

What is the primary hemodynamic effect of Ventricular Septal Defect

Right ventricular pressure overload

What pressure load occurs in a large Ventricular Septal Defect

Asymptomatic

What is the classic clinical presentation of a small Ventricular Septal Defect

Heart failure at 6 to 8 weeks, diaphoresis with feeding, failure to thrive, and recurrent respiratory tract infections

What are four classic symptoms of a large Ventricular Septal Defect in an infant

Loud harsh holosystolic murmur

What is the characteristic murmur of Ventricular Septal Defect

Left middle parasternal border with or without a thrill

Where is the Ventricular Septal Defect murmur loudest and what finding may accompany it

Ventricular hypertrophy

What ECG abnormality can be seen in large Ventricular Septal Defect lesions

Cardiomegaly, increased pulmonary vascular markings, and dilated main pulmonary artery

What are three chest X-ray findings in moderate to large Ventricular Septal Defects

Most common cardiac malformation

What is the epidemiologic status of Ventricular Septal Defect among all congenital heart diseases

Perimembranous

What is the most common anatomical type of Ventricular Septal Defect

Down syndrome

Which chromosomal disorder is inlet Ventricular Septal Defect specifically associated with

Pulmonary hypertension leading to Eisenmenger syndrome

What is the natural history risk of a large Ventricular Septal Defect

Heart failure medications

What is the initial medical management for symptomatic Ventricular Septal Defect

Surgical or device closure

What definitive therapies are indicated for Ventricular Septal Defect

Small Ventricular Septal Defect can have a louder murmur

What is the paradoxical exam clue regarding Ventricular Septal Defect murmur intensity and size

Muscular Ventricular Septal Defect

Which type of Ventricular Septal Defect is most likely to close spontaneously

Primum ASD plus inlet VSD plus common AV valve

What three structural anomalies define Atrioventricular Septal Defect

Multichamber volume overload and increased pulmonary blood flow

What is the primary hemodynamic effect of Atrioventricular Septal Defect

Heart failure in infancy, failure to thrive, and recurrent respiratory tract infections

What are three classic clinical presentations of Atrioventricular Septal Defect

Holosystolic murmur at the left lower parasternal border

What murmur type and location are characteristic of Atrioventricular Septal Defect

Superior or left axis deviation, first degree AV block, right ventricular hypertrophy, right bundle branch block, with or without left ventricular hypertrophy

What five ECG findings are associated with Atrioventricular Septal Defect

Multichamber cardiomegaly, increased pulmonary vascular markings, and prominent main pulmonary artery

What three chest X-ray findings are seen in Atrioventricular Septal Defect

Down syndrome or Trisomy 21

Which genetic disorder is Atrioventricular Septal Defect most strongly associated with

Endocardial cushion defect

What developmental defect category does Atrioventricular Septal Defect belong to

Heart failure within 12 months

Untreated complete Atrioventricular Septal Defect leads to what state and in what timeframe

Early pulmonary vascular disease

What natural history vascular complication occurs early in complete Atrioventricular Septal Defect

Heart failure medications followed by early surgical correction

What is the management timeline and strategy for complete Atrioventricular Septal Defect

Down syndrome plus heart failure infant plus left or superior axis deviation

What is the classic clinical exam clue for Atrioventricular Septal Defect

Aorta to pulmonary artery, left-to-right

What is the shunt connection and direction in Patent Ductus Arteriosus

Left ventricular volume overload

What is the primary hemodynamic effect of Patent Ductus Arteriosus on the left ventricle

Right ventricular pressure overload

What pressure load occurs in a large Patent Ductus Arteriosus

Heart failure, failure to thrive, and recurrent respiratory tract infections

What are three classic clinical findings of a large Patent Ductus Arteriosus in infants

Bounding pulses and wide pulse pressure

What are two classic peripheral vascular findings in Patent Ductus Arteriosus

Continuous murmur

What is the classic murmur heard in Patent Ductus Arteriosus

Left infraclavicular area or left upper parasternal border

What are two locations where the continuous murmur of Patent Ductus Arteriosus is best heard

Left ventricular hypertrophy

What is the characteristic ECG finding in Patent Ductus Arteriosus

Right ventricular hypertrophy

What ECG finding can develop in Patent Ductus Arteriosus if pulmonary hypertension is present

Cardiomegaly, left atrial and left ventricular enlargement, dilated main pulmonary artery, and increased pulmonary vascular markings

What four chest X-ray findings are characteristic of Patent Ductus Arteriosus

Female

Which gender has a higher predisposition to Patent Ductus Arteriosus

Pulmonary hypertension leading to Eisenmenger syndrome and infective endarteritis

What are two natural history risks of a large untreated Patent Ductus Arteriosus

Transcatheter coil or plug closure

What is the preferred management for Patent Ductus Arteriosus

Ligation or transection

What surgical options are performed for Patent Ductus Arteriosus if device closure is unsuitable

Continuous murmur plus bounding pulses plus wide pulse pressure

What is the key clinical exam triad for Patent Ductus Arteriosus

Aortic narrowing, usually juxtaductal

What is the core anatomical defect and its typical location in Coarctation of the Aorta

Left ventricular pressure overload

What is the primary hemodynamic load caused by Coarctation of the Aorta

Left ventricular hypertrophy

What structural cardiac adaptation occurs in response to the pressure overload in Coarctation of the Aorta

Upper-extremity hypertension plus weak or delayed femoral pulses

What classic presentation is seen in an older child with Coarctation of the Aorta

Shock as the patent ductus arteriosus closes

How does a critical infant with Coarctation of the Aorta classically present

Systolic ejection murmur

What is the characteristic murmur of Coarctation of the Aorta

Left interscapular back

Where can the murmur of Coarctation of the Aorta often be heard other than the anterior chest

Right ventricular hypertrophy

What ECG finding is characteristic of Coarctation of the Aorta in an infant

Left ventricular hypertrophy

What ECG finding is characteristic of Coarctation of the Aorta in older children

Rib notching

What classic radiological sign is seen on chest X-ray in older children with Coarctation of the Aorta

Cardiomegaly or pulmonary edema

What chest X-ray findings can be seen in infants with critical Coarctation of the Aorta

Turner syndrome

Which chromosomal syndrome is Coarctation of the Aorta strongly associated with

Bicuspid aortic valve

Which valvular abnormality is a key cardiac association of Coarctation of the Aorta

Left ventricular failure

What is a major natural history complication of untreated Coarctation of the Aorta

Hypertension complications

What systemic vascular complications can occur in the natural history of Coarctation of the Aorta

Prostaglandin E1 stabilization followed by repair

What is the initial medical and subsequent management for a critical infant with Coarctation of the Aorta

Surgery

What is the gold standard management for Coarctation of the Aorta, particularly in young patients

Arm hypertension plus weak or delayed femoral pulse

What is the classic clinical exam clue for Coarctation of the Aorta

Left ventricular outflow obstruction, usually valvar

What is the core structural defect and site in Aortic Stenosis

Left ventricular pressure overload

What is the main hemodynamic effect of Aortic Stenosis on the left ventricle

Left ventricular hypertrophy

What ventricular adaptation occurs in response to the pressure overload of Aortic Stenosis

Exertional chest pain, syncope, and fatigue

What three classic clinical symptoms indicate severe Aortic Stenosis

Sudden death

What sudden catastrophic event is a primary risk of severe untreated Aortic Stenosis

Harsh midsystolic ejection murmur

What is the characteristic murmur heard in Aortic Stenosis

Right upper parasternal border

Where is the Aortic Stenosis murmur loudest on the chest

Radiates to the neck

Where does the murmur of Aortic Stenosis classically radiate

Systolic thrill

What tactile sensation can be felt on the chest wall in Aortic Stenosis

Left ventricular hypertrophy with or without strain

What ECG findings are characteristic of Aortic Stenosis

Usually normal

How does the heart size on chest X-ray typically appear in Aortic Stenosis

Poststenotic ascending aortic dilation

What classic vascular finding is seen on chest X-ray in Aortic Stenosis

Williams syndrome

Which genetic syndrome is supravalvar Aortic Stenosis specifically associated with

Progressive

How is the natural history of Aortic Stenosis generally characterized

Prostaglandin E1 stabilization

What is the initial treatment for critical neonatal Aortic Stenosis

Balloon valvuloplasty

What is the procedure of choice for severe or critical Aortic Stenosis

Exertional syncope plus right upper parasternal border murmur

What is the classic clinical exam clue for Aortic Stenosis

Right ventricular outflow obstruction, usually valvar

What is the core structural defect and site of Pulmonary Stenosis

Right ventricular pressure overload

What is the main hemodynamic effect of Pulmonary Stenosis on the right ventricle

Right ventricular hypertrophy

What ventricular adaptation occurs in response to the pressure overload of Pulmonary Stenosis

Mild

Which severity of Pulmonary Stenosis is typically asymptomatic

Exertional dyspnea and exertional chest pain

What two symptoms are classic for severe Pulmonary Stenosis

Cyanosis

How may a critical neonate with Pulmonary Stenosis present clinically

Systolic ejection murmur

What is the characteristic murmur of Pulmonary Stenosis