[02.20a] CM - Congenital Heart Diseases_ Acyanotic V2.pdf

Birth defects

What is the leading cause of infant mortality?

Congenital heart disease

What is the most common birth defect?

An anatomic malformation of the heart and/or its vessels occurring during intrauterine development

What is the definition of Congenital Heart Disease?

1 percent

What is the birth prevalence of Congenital Heart Disease?

23 per 1000 live births

What is the prevalence of clinically severe Congenital Heart Disease conditions?

48 per 1000 live births

What is the prevalence of milder or simpler Congenital Heart Disease conditions?

Congenital or acquired

What are the two general classifications of heart disease based on origin?

Assess whether cyanosis is present or absent

What is the first major step in the initial evaluation of suspected Congenital Heart Disease?

Physical examination and pulse oximetry

What two methods are used to determine and support the assessment of cyanosis in suspected Congenital Heart Disease?

Chest X-ray

What imaging modality is used second in the evaluation flowchart for suspected Congenital Heart Disease?

Evaluate pulmonary vascular markings and determine if pulmonary blood flow is increased, normal, or decreased

What are the two main purposes of obtaining a chest X-ray in a suspected Congenital Heart Disease patient?

ECG

What test can be used to determine if there is evidence of right or left ventricular hypertrophy in suspected Congenital Heart Disease?

Shunt lesions and obstructive lesions

What are the two main pathophysiological categories of acyanotic Congenital Heart Disease?

Volume overload

What hemodynamic consequence do shunt lesions cause in acyanotic Congenital Heart Disease?

Pressure overload

What hemodynamic consequence do obstructive lesions cause in acyanotic Congenital Heart Disease?

Shunt lesions

Which type of lesions are indicated when a chest X-ray shows increased pulmonary blood flow?

Tachypnea or breathing difficulty

What common respiratory symptoms are seen in patients with shunt lesions due to volume overload or pulmonary congestion?

Obstructive lesions

Which type of lesions are indicated when a chest X-ray shows normal or decreased pulmonary blood flow in an acyanotic patient with minimal congestion?

Ejection murmur

What clinical finding on physical exam points towards obstructive lesions in acyanotic Congenital Heart Disease?

History and physical examination

What two clinical actions are required to begin the recognition of Congenital Heart Disease?

Heart failure signs and symptoms and presence of murmur

What are the two most common clinical triggers to investigate a child for Congenital Heart Disease?

Intermittency of feeding, diaphoresis, tachypnea or dyspnea, and failure to thrive

What are four classic signs of heart failure in infants with Congenital Heart Disease?

Arrhythmia, hypertension, syncope, and chest pain

What are four less common clinical presentations of acyanotic Congenital Heart Disease?

Superior vena cava and inferior vena cava

From what two major systemic vessels does deoxygenated blood drain into the right atrium?

Tricuspid valve

Through which valve does blood flow from the right atrium to the right ventricle?

Pulmonary valve

Through which valve does blood flow from the right ventricle into the pulmonary artery?

Lungs

Where does deoxygenated blood go from the pulmonary artery to become oxygenated?

Pulmonary veins

Through which vessels does oxygenated blood travel from the lungs to the left atrium?

Mitral valve

Through which valve does blood flow from the left atrium to the left ventricle?

Aortic valve

Through which valve does blood flow from the left ventricle into the aorta?

Systemic circulation

Where does blood go from the aorta?

Shunt lesions

Which group of acyanotic heart diseases produce volume overload?

Atrial Septal Defect, Ventricular Septal Defect, Atrioventricular Septal Defect, and Patent Ductus Arteriosus

What are the four common left-to-right shunt lesions discussed in the text?

Atrial Septal Defect

What is an abnormal, fixed opening that occurs in any portion of the atrial septum allowing communication of blood between the left and right atria?

Primum, secundum, and sinus venosus

What are the three types of Atrial Septal Defects named after the embryonal septal structures that failed to develop normally?

Secundum

Which type of Atrial Septal Defect represents 7 percent of all congenital heart defects?

Sporadic

How are the majority of cases of Atrial Septal Defect inherited?

Autosomal dominant

What mode of inheritance can occur in some familial cases of Atrial Septal Defect?

Holt-Oram syndrome

What specific autosomal dominant syndrome presents with hypoplastic or absent thumbs, phocomelia, first degree AV block on ECG, and an Atrial Septal Defect?

Females

In which sex is Atrial Septal Defect more commonly seen?

Center at the area of the fossa ovalis

Where is the defect located in an ostium secundum Atrial Septal Defect?

Near the AV valves

Where is the defect located in an ostium primum Atrial Septal Defect?

Near the entry of the superior vena cava or the inferior vena cava to the right atrium

Where is the defect located in a sinus venosus Atrial Septal Defect?

Ostium secundum

What is the most common type of Atrial Septal Defect, accounting for 50 to 70 percent of all cases?

Ostium primum

Which type of Atrial Septal Defect accounts for about 30 percent of all cases?

Sinus venosus

Which type of Atrial Septal Defect accounts for around 10 percent of all cases?

Entry of the superior vena cava into the right atrium

What is the most common specific presentation site for a sinus venosus Atrial Septal Defect?

Volume overload of the right ventricle and hypertrophy of the right atrium and right ventricle

What are the main hemodynamic consequences of an Atrial Septal Defect?

Left side to the right side

In which direction does blood naturally flow through an Atrial Septal Defect because of the higher pressure on the left side of the heart?

Right side of the heart

Which side of the heart becomes overloaded over time due to the constant left-to-right shunting in an Atrial Septal Defect?

Asymptomatic

How do most patients with an Atrial Septal Defect clinically present initially?

Second decade of life

Around what age are asymptomatic Atrial Septal Defect patients typically diagnosed via physical examination or finding cardiomegaly?

Tachypnea, failure to thrive, and respiratory infections

What are three symptoms of congestive heart failure that can occur in young children with large Atrial Septal Defects?

Exercise intolerance, dyspnea, and easy fatigability

What are three common symptoms seen in older children with large Atrial Septal Defects?

Mild precordial bulge and right ventricular systolic lift

What two palpable physical exam signs may be present in patients with large Atrial Septal Defect shunts?

Wide, fixed, split S2

What is the classic heart sound finding on auscultation in a significant Atrial Septal Defect?

Splitting is present during all phases of respiration

What does a fixed split S2 mean?

Right side of the heart is volume-loaded

Why is a split S2 heard in an Atrial Septal Defect?

Inspiration

Normally, during which phase of respiration is a split S2 heard?

Right ventricular volume increases, delaying closure of the pulmonary valve

What normal physiological mechanism causes S2 splitting during deep inspiration?

Systolic ejection murmur

What type of murmur is heard at the left upper parasternal border in Atrial Septal Defect due to increased flow across the RV outflow tract and relative pulmonary stenosis?

Short, rumbling, mid-diastolic murmur

What type of murmur may be heard at the left middle parasternal border in Atrial Septal Defect due to increased volume across the tricuspid valve?

Distended neck veins, edema, and hepatomegaly

What are three physical signs of right ventricular failure in the late stage of Atrial Septal Defect?

Normal

What is the typical chest X-ray finding in a patient with a small Atrial Septal Defect?

Right ventricular hypertrophy, dilated main pulmonary artery, and increased pulmonary blood flow

What three findings are typically seen on chest X-ray in a significant Atrial Septal Defect?

Prominent right cardiac border and increased pulmonary vascular markings especially on the right pulmonary artery segment

What does a posterior-anterior chest X-ray view show in significant Atrial Septal Defect?

Retrosternal fullness

What finding on a lateral chest X-ray view indicates right ventricular hypertrophy in an Atrial Septal Defect?

Jug-handle-appearance

What classical radiographic sign of Atrial Septal Defect is formed by a dilated right pulmonary artery, dilated right atrium, and right ventricular volume overload on chest X-ray?

Dilated right pulmonary artery

What structure forms the handle of the jug in the classical jug-handle-appearance on chest X-ray?

Dilated right atrium and right ventricle

What structures form the body of the jug in the classical jug-handle-appearance on chest X-ray?

Dilated main pulmonary artery, right ventricular dilatation with uplifted apex, dilated right descending pulmonary artery, and right atrial enlargement

What are the four components of the typical jug-handle-appearance on chest X-ray?

Right atrial enlargement and right ventricular hypertrophy

What chamber enlargements are visible on an ECG for Atrial Septal Defect?

Normal or right-axis deviation

What axis deviation is typically seen on an ECG for Atrial Septal Defect?

Right bundle branch block

What conduction block is commonly seen on ECG in Atrial Septal Defect?

First degree AV block

What rhythm disturbance can be seen on ECG in Atrial Septal Defect?

40 percent

What percentage of small ostium secundum Atrial Septal Defects close spontaneously by age 4?

Greater than 80 percent

What is the chance of spontaneous closure for Atrial Septal Defects measuring between 3 to 8 mm before 1 1/2 years of age?

Atrial Septal Defect diameter greater than 8 mm

What size threshold of Atrial Septal Defect rarely closes spontaneously?

Beyond 4 years of age

Beyond what age is spontaneous closure of an Atrial Septal Defect unlikely?

Congestive heart failure, pulmonary hypertension, and Eisenmengerization

What three serious long-term conditions can develop in the second to third decade of life from an untreated large Atrial Septal Defect?

Atrial arrhythmias, cerebrovascular accidents, and infective endocarditis

What are three potential complications of untreated Atrial Septal Defects?

Infective endocarditis

Which complication of Atrial Septal Defect is noted to be rare?

All symptomatic patients

In which group of patients is a transcatheter device or surgery advised for Atrial Septal Defect?

Control symptoms of heart failure

What is the limited role of medical anti-heart failure medications in Atrial Septal Defect management?

Transcatheter device closure

What is the preferred non-surgical method for closing an Atrial Septal Defect?

Secundum

Which type of Atrial Septal Defect is primarily indicated for transcatheter device closure?

Presence of a large shunt causing hemodynamic instability

What is the primary indication for closing an Atrial Septal Defect?

Defect size of 5 mm or more, Qp to Qs ratio of 1.5 or more, and enough rim or septal tissue of 4 to 5 mm

What are three specific criteria regarding defect size, shunt significance, and rim size for Atrial Septal Defect device closure?

Diastolic flow rumble in the tricuspid area, ECG right ventricular hypertrophy, and chest X-ray cardiomegaly with increased pulmonary vascular markings

What clinical, electrocardiographic, and radiographic findings serve as indicators of a significant left-to-right shunt for Atrial Septal Defect closure?

Right ventricular enlargement and/or paradoxical septal motion

What two echocardiographic findings indicate the need for Atrial Septal Defect device closure?

Avoiding bypass, smaller incision or puncture sites, and faster recovery

What are three main advantages of transcatheter device closure over surgical closure for Atrial Septal Defect?

Possibility of spontaneous closure

Why is the optimal timing of transcatheter device closure in secundum Atrial Septal Defects not entirely clear?

At least 15 kg

What is the recommended minimum weight that simplifies the procedure and offers technical advantages for Atrial Septal Defect transcatheter closure?

Surgical closure

What type of closure is indicated when Atrial Septal Defect device closure is not considered appropriate?

Sinus venosus, coronary sinus, primum, and secundum with inadequate rims

What are four types or conditions of Atrial Septal Defects that must be closed surgically instead of with a device?

Ventricular Septal Defect

What is the most common congenital cardiac malformation, accounting for 25 to 40 percent of all Congenital Heart Disease cases?

Interventricular septum

Along what structure is a Ventricular Septal Defect located, allowing communication between the left and right ventricles?

Perimembranous, muscular, inlet, and outlet

What are the four anatomic types of Ventricular Septal Defects?

Perimembranous

What is the most common type of Ventricular Septal Defect, located at the base of the heart?

Posteroinferior position anterior to the septal leaflet of the tricuspid valve

Where is a perimembranous Ventricular Septal Defect anatomically located in relation to the tricuspid valve?