The most common genetic cause of congenital heart disease is:

A) Trisomy 13

B) Turner syndrome

C) Trisomy 21

D) DiGeorge syndrome

C. Trisomy 21

Congenital heart disease in trisomy 21 most often affects which structure derived largely from the second heart field?

A) Aortic arch

B) AV septum

C) Pulmonary valve

D) Left ventricle

B. AV septum

A patient with a congenital bicuspid aortic valve is most likely to have a mutation in:

A) NOTCH1

B) JAG1

C) NKX2-5

D) TBX5

A. NOTCH1

Mutations in NOTCH2 and JAG1 are classically associated with:

A) Coarctation

B) PDA

C) Tetralogy of Fallot

D) ASD

C. Tetralogy of Fallot

The force generated by cardiac muscle depends most directly on the:

A) Number of nuclei

B) Distance each sarcomere contracts

C) Calcium stores per atrium

D) Valve recoil speed

B. Distance each sarcomere contracts

Excessive ventricular dilation reduces actin-myosin overlap, sharply lowering contractile force and predisposing to:

A) Bradycardia

B) Heart failure

C) Pericarditis

D) Cyanosis

B. Heart failure

Compared with ventricular myocytes, atrial myocytes are more haphazardly arranged and generate:

A) Stronger forces

B) Weaker forces

C) Equal forces

D) No force

B. Weaker forces

The coordinated beating of cardiac myocytes depends primarily on:

A) Purkinje fat pads

B) Intercalated discs

C) Chordae tendineae

D) Fibrous trigones

B. Intercalated discs

Abnormal spatial distribution of cardiac gap junctions most directly predisposes to:

A) Valve calcification

B) Arrhythmia and heart failure

C) Aortic coarctation

D) Pericardial tamponade

B. Arrhythmia and heart failure

Cardiac valves are lined by:

A) Mesothelium

B) Endothelium

C) Epithelium

D) Myocardium

B. Endothelium

Valvular interstitial cells are chiefly responsible for synthesizing:

A) Troponin filaments

B) Extracellular matrix

C) Action potentials

D) Coronary endothelium

B. Extracellular matrix

In addition to producing matrix, valvular interstitial cells also express matrix-____ enzymes.

A) building

B) stiffening

C) degrading

D) sealing

C. degrading

Competence of the atrioventricular valves depends on proper function of the leaflets, tendinous cords, and:

A) Coronary sinus

B) Papillary muscles

C) AV node

D) Trabeculae carneae

B. Papillary muscles

Which pathologic valvular change most directly weakens leaflets by disrupting structural support?

A) Nodular calcification

B) Fibrotic thickening

C) Collagen damage

D) Endothelial hyperplasia

C. Collagen damage

Which of the following is a classic pathologic change of cardiac valves?

A) Nodular calcification

B) Purkinje hypertrophy

C) Coronary thrombosis

D) Septal rupture

A. Nodular calcification

Cardiac myocytes rely almost exclusively on which pathway for energy production?

A) Anaerobic glycolysis

B) Oxidative phosphorylation

C) Pentose pathway

D) Beta oxidation only

B. Oxidative phosphorylation

Coronary arteries running on the external surface of the heart are called:

A) Intramural coronaries

B) Endocardial coronaries

C) Epicardial coronaries

D) Thebesian coronaries

C. Epicardial coronaries

Once coronary arteries penetrate the myocardium, they are termed:

A) Intramural arteries

B) Marginal arteries

C) Septal veins

D) Endocardial sinuses

A. Intramural arteries

The major named branches of the left anterior descending artery are the:

A) Marginal branches

B) Diagonal branches

C) Septal veins

D) Circumflex branches

B. Diagonal branches

The major named branches of the left circumflex artery are the:

A) Diagonal branches

B) Marginal branches

C) Infundibular branches

D) Anastomotic branches

B. Marginal branches

Most blood flow to the myocardium occurs during:

A) Atrial systole

B) Ventricular systole

C) Ventricular diastole

D) Isovolumic contraction

C. Ventricular diastole

Cardiac stem cells proliferate at a very ____ rate, with the greatest activity in neonates.

A) rapid

B) variable

C) slow

D) absent

C. slow

Bulging of the basal ventricular septum into the LV outflow tract is termed:

A) Muscular ridge

B) Sigmoid septum

C) Septal aneurysm

D) Dynamic shelf

B. Sigmoid septum

Congestive heart failure is present when the heart cannot meet tissue ____ or can do so only at elevated filling pressure.

A) oxygen saturation

B) metabolic demands

C) venous tone

D) coronary reserve

B. metabolic demands

Systolic heart failure results from progressive deterioration of myocardial ____ function.

A) contractile

B) endocrine

C) conductive

D) valvular

A. contractile

Diastolic heart failure is recognized as an inability of the heart chamber to expand and fill sufficiently during:

A) systole

B) repolarization

C) diastole

D) ejection

C. diastole

Cardiac hypertrophy requires increased protein synthesis mainly to assemble additional:

A) capillaries

B) sarcomeres

C) valves

D) fibroblasts

B. sarcomeres

The main structural result of volume-overload hypertrophy is:

A) ventricular dilation

B) septal thinning only

C) valve calcification

D) atrial standstill

A. ventricular dilation

In dilated hearts, the best measure of hypertrophy is:

A) wall thickness

B) ejection fraction

C) heart weight

D) chamber pressure

C. heart weight

Myocyte hypertrophy is not accompanied by a proportional increase in:

A) capillary numbers

B) mitochondria

C) protein synthesis

D) myofilaments

A. capillary numbers

Oxygen consumption by the hypertrophied heart is typically:

A) Decreased

B) Elevated

C) Unchanged

D) Intermittent

B. Elevated

At autopsy, hearts from patients with chronic congestive heart failure are generally:

A) Small and rigid

B) Heavy and dilated

C) Light and thick-walled

D) Small and hyperdynamic

B. Heavy and dilated

In CHF, the ventricular walls at autopsy may appear relatively:

A) Calcified

B) Thin-walled

C) Nodular

D) Septated

B. Thin-walled

Aerobic exercise is most associated with what cardiac adaptation?

A) Pressure-load hypertrophy

B) Volume-load hypertrophy

C) Fibrotic atrophy

D) Septal calcification

B. Volume-load hypertrophy

Compared with static exercise, aerobic exercise is more likely to increase:

A) Valve thickness

B) Capillary density

C) Resting blood pressure

D) Resting heart rate

B. Capillary density

Aerobic exercise causes a more physiologic adaptation with improved oxygen delivery to myocardium, including increased capillary density.

Regular aerobic training is often associated with decreased resting:

A) HR and BP

B) EF and preload

C) SV and CO

D) RV and LV mass

A. HR and BP

training makes the cardiovascular system more efficient at rest.

Static exercise such as weight lifting is most associated with:

A) Volume hypertrophy

B) Pressure hypertrophy

C) Atrial dilation only

D) Valve prolapse

B. Pressure hypertrophy

Forward failure in CHF is best defined by:

A) Venous pooling and perfusion

B) Decreased CO and perfusion

C) Pulmonary fibrosis and perfusion

D) AV valve calcification and perfusion

B. Decreased CO and perfusion

Forward failure = the heart cannot pump enough blood forward.

Backward failure in CHF is characterized primarily by:

A) Arterial vasospasm

B) Venous blood pooling

C) Coronary thrombosis

D) Conduction block

B. Venous blood pooling

Backward failure can produce which edema pattern?

A) Pulmonary or peripheral edema

B) Cerebral edema or peripheral edema

C) Myxedema or peripheral edema

D) Lymphatic edema or peripheral edema

A. Pulmonary or peripheral edema

The most common cause of right-sided heart failure is:

A) Primary RV infarction

B) Left-sided heart failure

C) Pulmonary embolism

D) Tricuspid stenosis

B. Left-sided heart failure

Generalized massive edema is termed:

A) Anasarca

B) Ascites

C) Hydrothorax

D) Lymphedema

A. Anasarca

Which two organs are prominently affected in right-sided heart failure?

A) Spleen and pancreas

B) Kidney and brain

C) Thyroid and thymus

D) Adrenals and colon

B. Kidney and brain

Which condition is the most common cause of left-sided heart failure?

A) Hyperthyroidism

B) Ischemic heart disease

C) Tetralogy of Fallot

D) Pulmonary fibrosis

B. Ischemic heart disease

A common major cause of left-sided CHF besides ischemic disease is:

A) Hypertension

B) Renal agenesis

C) Pericarditis

D) Atrial septal defect

A. Hypertension

Left-sided CHF may also result from disease of which valves?

A) Tricuspid and pulmonic

B) Aortic and mitral

C) Mitral and tricuspid

D) Aortic and pulmonic

B. Aortic and mitral

Hemosiderin-laden macrophages in the lung are classic markers of prior:

A) Pleuritis

B) Pulmonary edema

C) Asthma

D) Sarcoidosis

B. Pulmonary edema

Hemosiderin-laden macrophages found in CHF are commonly called:

A) Aschoff cells

B) Heart failure cells

C) Foam cells

D) Anitschkow cells

B. Heart failure cells

As CHF worsens, progressive pulmonary edema commonly causes:

A) Hemoptysis and stridor

B) Orthopnea and PND

C) Cyanosis and clubbing

D) Bradycardia and syncope

B. Orthopnea and PND

Atrial fibrillation is best described as what type of atrial activity?

A) Slow coordinated contraction

B) Uncoordinated chaotic contraction

C) Fixed mechanical standstill

D) Hyperdynamic ejection

B. Uncoordinated chaotic contraction

A reduced ejection fraction decreases perfusion to which organ, thereby activating RAAS?

A) Liver

B) Kidney

C) Brain

D) Spleen

B. Kidney

Mutations in which transcription factors are associated with atrial and ventricular septal defects?

A) GATA4, TBX5, NKX2-5

B) MYOD, PAX6, WT1

C) FBN1, ELN, CFTR

D) RET, KIT, APC

A. GATA4, TBX5, NKX2-5

Mutations in which signaling pathway are associated with congenital heart defects such as bicuspid aortic valve and tetralogy?

A) Hedgehog

B) Wnt

C) Notch

D) TGF-beta

C. Notch

Deletion 22q11.2 occurs in up to half of patients with:

A) Turner syndrome

B) DiGeorge syndrome

C) Marfan syndrome

D) Noonan syndrome

B. DiGeorge syndrome

In DiGeorge syndrome, abnormal development involves the fourth branchial arch and derivatives of which pouches?

A) First and second

B) Second and third

C) Third and fourth

D) Fourth and fifth

C. Third and fourth

The mnemonic CATCH-22 in DiGeorge syndrome includes all except:

A) Cardiac abnormality

B) Thymic aplasia

C) Cleft palate

D) Hyperkalemia

D. Hyperkalemia

Which transcription factor gene is deleted in many cases of DiGeorge syndrome?

A) TBX1

B) GATA4

C) NKX2-5

D) NOTCH1

A. TBX1

TBX1 is important for neural crest migration and expansion of progenitors in which heart field?

A) First heart field

B) Second heart field

C) Proepicardium

D) Septum secundum

B. Second heart field

Important chromosomal aneuploidies associated with congenital heart disease include Turner syndrome and trisomies:

A) 8, 9, and 10

B) 11, 12, and 13

C) 13, 18, and 21

D) 15, 16, and 17

C. 13, 18, and 21

Most structural congenital heart defects can be grouped into which three categories?

A) Stenosis, rupture, hypertrophy

B) Left-to-right, right-to-left, obstruction

C) Cyanotic, acyanotic, valvular

D) Septal, valvular, conduction

B. Left-to-right, right-to-left, obstruction

A shunt in congenital heart disease is an abnormal:

A) Valve thickening

B) Chamber enlargement

C) Communication

D) Pressure gradient

C. Communication

Once irreversible pulmonary hypertension develops in a left-to-right shunt, the defect is generally considered:

A) Benign

B) Reversible

C) Irreparable

D) Compensated

C. Irreparable

Obstructive congenital heart disease results from abnormal:

A) Narrowing

B) Dilation

C) Fibrosis

D) Cyanosis

A. Narrowing

A complete congenital obstruction is called:

A) Stenosis

B) Atresia

C) Coarctation

D) Hypoplasia

B. Atresia

The most common overall category of congenital heart disease is:

A) Right-to-left shunts

B) Obstructive lesions

C) Left-to-right shunts

D) Valve atresias

C. Left-to-right shunts

Most atrial septal defects are usually asymptomatic until:

A) Infancy

B) Childhood

C) Adolescence

D) Adulthood

D. Adulthood

The septum primum is best described as a crescent-shaped membranous ingrowth between the:

A) Ventricles

B) Atria

C) Great arteries

D) AV valves

B. Atria

During fetal development, the ostium primum allows blood flow from the:

A) Left atrium to right

B) Right atrium to left

C) Right ventricle to left

D) Pulmonary artery to aorta

B. Right atrium to left

Before the septum primum fully closes the ostium primum, it develops a second opening called the:

A) Foramen ovale

B) Septum secundum

C) Ostium secundum

D) Ductus venosus

C. Ostium secundum

As the septum secundum grows, it leaves a small opening called the:

A) Ostium primum

B) Foramen ovale

C) Sinus venosus

D) AV canal

B. Foramen ovale

The flap of the foramen ovale opens when pressure is greater in the:

A) Left atrium

B) Right atrium

C) Left ventricle

D) Pulmonary vein

B. Right atrium

At birth, lung expansion lowers pulmonary vascular pressure, causing right atrial pressure to fall below left atrial pressure and the foramen ovale to:

A) Widen

B) Calcify

C) Close

D) Shunt rightward

C. Close

The most common type of ASD is:

A) Primum ASD

B) Sinus venosus ASD

C) Secundum ASD

D) Coronary sinus ASD

C. Secundum ASD

A murmur in ASD is usually due to excessive flow through the pulmonary valve and/or:

A) Aortic root

B) The ASD itself

C) Mitral valve

D) Coronary sinus

B. The ASD itself

Mortality is generally low in:

A) VSDs

B) Tetralogy

C) ASDs

D) Truncus arteriosus

C. ASDs

The most common form of congenital heart disease overall is:

A) ASD

B) PDA

C) Coarctation

D) VSD

D. VSD

VSDs are incomplete closures of the ventricular septum that allow blood communication between the:

A) Atria

B) Ventricles

C) Aorta and PA

D) Veins

B. Ventricles

VSDs are classified mainly by their:

A) Size and location

B) Murmur pitch

C) Oxygen saturation

D) Age at diagnosis

A. Size and location

A membranous VSD is located in the:

A) Pulmonary trunk

B) Atrial septum

C) Intraventricular septum

D) Mitral annulus

C. Intraventricular septum

An infundibular VSD is found within the muscular septum or just below the:

A) Aortic valve

B) Pulmonary valve

C) Tricuspid valve

D) Mitral valve

B. Pulmonary valve

Pediatric VSDs that become clinically apparent are often associated with other anomalies such as:

A) Mitral prolapse

B) Tetralogy of Fallot

C) ASD secundum

D) Coarctation

B. Tetralogy of Fallot

Large unclosed VSDs almost universally lead over time to irreversible:

A) Aortic stenosis

B) Pulmonary vascular disease

C) Tricuspid atresia

D) Myocarditis

B. Pulmonary vascular disease

The long-term result of severe pulmonary vascular disease from an unclosed VSD is often shunt reversal, cyanosis, and:

A) Recovery

B) Death

C) Bradycardia

D) Valve closure

B. Death

The ductus arteriosus arises from the:

A) Aorta

B) Pulmonary artery

C) Left ventricle

D) SVC

B. Pulmonary artery

Which change promotes ductus arteriosus closure after birth?

A) Lower oxygenation

B) Increased prostaglandin E2

C) Increased oxygenation

D) Higher pulmonary resistance

C. Increased oxygenation

After birth, the newborn starts breathing, so blood oxygen levels rise. Higher oxygen causes the ductus arteriosus smooth muscle to constrict and close.

A second factor promoting ductus arteriosus closure is decreased:

A) Systemic vascular resistance

B) Pulmonary vascular resistance

C) Left atrial pressure

D) Heart rate

B. Pulmonary vascular resistance

A third factor promoting ductus arteriosus closure is declining local levels of:

A) Prostaglandin E2

B) Nitric oxide

C) Endothelin

D) Angiotensin II

A. Prostaglandin E2

After complete closure, the ductus arteriosus becomes the:

A) Ligamentum venosum

B) Ligamentum arteriosum

C) Coronary ligament

D) Annulus fibrosus

B. Ligamentum arteriosum

PDA classically produces what type of murmur?

A) Opening snap

B) Machinery-like murmur

C) Late systolic click

D) Low diastolic rumble

B. Machinery-like murmur

Which lesion most commonly increases pulmonary outflow volume without greatly increasing pulmonary pressure early on?

A) ASD

B) VSD

C) PDA

D) Tetralogy

A. ASD

Because the shunt is from left atrium → right atrium, the pressure difference is relatively small. So esp early on, not much is happening.

Which pair typically increases both pulmonary blood flow and pulmonary pressure?

A) ASD and PFO

B) VSD and PDA

C) ASD and VSD

D) PDA and coarctation

B. VSD and PDA

Which septal structure develops first as a crescent-shaped ingrowth between the atria?

A) Septum secundum

B) Septum primum

C) Endocardial cushion

D) Foramen ovale

B. Septum primum

Which opening is continuous with the ostium secundum?

A) Ductus arteriosus

B) Coronary sinus

C) Foramen ovale

D) Ostium primum

C. Foramen ovale

Which congenital category best describes ASD, VSD, and PDA before pulmonary hypertension becomes irreversible?

A) Right-to-left shunts

B) Left-to-right shunts

C) Obstructive lesions

D) Cyanotic lesions

B. Left-to-right shunts

A cyanotic infant with Tetralogy of Fallot classically has a heart that is:

A) Small and globular

B) Enlarged and boot-shaped

C) Narrow and elongated

D) Calcified and rigid

B. Enlarged and boot-shaped

The clinical severity of Tetralogy of Fallot depends mainly on the severity of:

A) Aortic override

B) RV hypertrophy

C) Subpulmonary stenosis

D) Atrial septal defect

C. Subpulmonary stenosis

Why does subpulmonary stenosis largely determine Tetralogy physiology?

A) It sets blood-flow direction

B) It closes the ductus

C) It fixes atrial pressure

D) It blocks coronaries

A. It sets blood-flow direction

Transposition of the great arteries produces which anatomic relationship?

A) Atrioventricular concordance

B) Ventriculoarterial discordance

C) AV septal deficiency

D) Left-to-right shunting

B. Ventriculoarterial discordance

Tricuspid atresia is best defined as complete occlusion of the:

A) Mitral annulus

B) Pulmonic outflow

C) Tricuspid valve orifice

D) Coronary sinus ostium

C. Tricuspid valve orifice

Embryologically, tricuspid atresia most directly results from unequal division of the:

A) Truncus arteriosus

B) AV canal

C) Septum secundum

D) Aortic sac

B. AV canal

The infantile form of coarctation usually features tubular hypoplasia of the arch:

A) Distal to a PDA

B) Proximal to a PDA

C) Distal to subclavian

D) At the aortic root

B. Proximal to a PDA

_____-type coarctation is classically a discrete, ridgelike narrowing of the aorta near the insertion of the ligamentum arteriosum, which is the remnant of the closed ductus arteriosus.

Adult

Coarctation of the aorta associated with a PDA usually presents:

A) Late in adulthood

B) Early in life

C) Only during pregnancy

D) Only after exercise

B. Early in life

Subaortic stenosis is usually associated with a prominent systolic:

A) Click

B) Murmur

C) Rub

D) Snap

B. Murmur

A palpable vibration may accompany subaortic stenosis as a:

A) Heave

B) Thrill

C) Knock

D) Gallop

B. Thrill

Subaortic stenosis is usually caused by dense fibrous tissue located:

A) Above the cusps

B) Below the cusps

C) Within the atrium

D) At the apex

B. Below the cusps

Compared with the RV, myocardial fibers are more circumferentially organized in a spiral pattern in the:

A) LV

B) RA

C) RV

D) LA

A. LV

Which valves have leaflets?

A) Semilunar valves

B) AV valves

B. AV valves

Which valves are described as having cusps?

A) AV valves

B) Semilunar valves

B. Semilunar valves

The most abundant cell type in cardiac valves is the valvular ____ cell.

A) endothelial

B) interstitial

C) nodal

D) mesothelial

B. interstitial

Which valvular structures are supported by chordae tendineae?

A) Semilunar cusps

B) AV leaflets

C) Aortic cusps

D) Pulmonic cusps

B. AV leaflets

Cardiac stem cells make up a greater proportion of normal cellularity in the:

A) ventricles

B) atria

C) septum

D) valves

B. atria

With aging, epicardial fat generally:

A) decreases

B) increases

C) calcifies only

D) disappears

B. increases

With aging, cardiac valves commonly develop:

A) fatty atrophy

B) fibrous thickening

C) myocyte hyperplasia

D) vascular proliferation

B. fibrous thickening

Mitral valve prolapse most directly increases pressure in the:

A) LV

B) LA

C) RV

D) aorta

B. LA

Age-related basophilic degeneration in myocytes reflects accumulation of a gray-blue byproduct of:

A) collagen synthesis

B) glycogen metabolism

C) fatty acid oxidation

D) calcium transport

B. glycogen metabolism

A pathologist examines hypertrophic cardiac myocytes. How do their nuclei typically appear?

A) Small and pyknotic

B) Enlarged

C) Fragmented

D) Binucleated only

B. Enlarged

In pressure-overload hypertrophy, new sarcomeres are added in:

A) Series

B) Parallel

C) Spirals

D) Clusters

B. Parallel

In volume-overload hypertrophy, new sarcomeres are added in:

A) Parallel

B) Rings

C) Series

D) Sheets

C. Series

Pressure-overload hypertrophy most characteristically produces:

A) Ventricular dilation

B) Thinner walls

C) Wall thickening

D) Atrial collapse

C. Wall thickening

Pressure overload makes the ventricle pump against increased resistance, so the muscle fibers add sarcomeres in parallel, producing concentric hypertrophy.

Volume-overload hypertrophy most characteristically produces:

A) Concentric thickening

B) Ventricular dilation

C) Septal calcification

D) Valve stenosis

B. Ventricular dilation

Volume overload means the ventricle has to handle too much blood volume, so it adapts by dilating/enlarging the chamber.

A patient with left-sided heart failure is at increased risk for atrial fibrillation mainly because LV dysfunction causes:

A) RV infarction

B) LA dilation

C) SA node fibrosis

D) PDA reopening

B. LA dilation

In left-sided heart failure, thrombus formation is especially likely in the:

A) Coronary sinus

B) Left atrial appendage

C) Right ventricle

D) Aortic root

B. Left atrial appendage

Far-advanced CHF may cause irritability, poor attention, and eventual coma from cerebral hypoperfusion. This is called:

A) Hepatic encephalopathy

B) Hypoxic encephalopathy

C) Uremic encephalopathy

D) Septic encephalopathy

B. Hypoxic encephalopathy

A hallmark hemodynamic finding in left-sided heart failure is increased:

A) RV systolic pressure only

B) LV end-diastolic pressure

C) Pulmonary valve area

D) Coronary sinus flow

B. LV end-diastolic pressure

In left-sided heart failure, reduced ejection fraction is the key finding in which type?

A) Diastolic failure

B) Systolic failure

C) Restrictive failure

D) High-output failure

B. Systolic failure

What is the main problem in diastolic left-sided heart failure?

A) Hyperdynamic ventricle

B) Stiff LV with impaired filling

C) Severe RV dilation

D) Reduced coronary flow only

B. Stiff LV with impaired filling

What is the ejection fraction in classic diastolic left-sided heart failure?

A) Elevated

B) Reduced

C) Normal

D) Absent

C. Normal

The LV is stiff and has trouble filling during diastole, but the percent of blood ejected from what it does receive is usually normal.

What is the most common underlying abnormality in diastolic left-sided heart failure?

A) COPD

B) Hypertension

C) Aortic dissection

D) Viral myocarditis

B. Hypertension

Low-flow symptoms such as cool extremities, cachexia, and confusion are more typical of:

A) Diastolic left HF

B) Systolic left HF

C) Isolated right HF

D) High-output HF

B. Systolic left HF

A patient with left-sided heart failure and pulmonary edema will most likely have which lung finding on auscultation?

A) Wheezes only

B) Rhonchi only

C) Crackles or rales

D) Pleural rub

C. Crackles or rales

Which heart sound is more associated with high left atrial pressure?

A) S1

B) S2

C) S3

D) S4

C. S3

Which heart sound is more associated with a stiff left ventricle?

A) S1

B) S2

C) S3

D) S4

D. S4

In which type of left-sided heart failure is an S4 most expected?

A) Systolic HF

B) Diastolic HF

C) High-output HF

D) Right-sided HF

B. Diastolic HF

S4 happens when the atrium contracts into a stiff, noncompliant ventricle. so its gonna be diastole

Cor pulmonale refers to isolated right-sided heart failure caused by:

A) Aortic stenosis

B) Lung disease or pulmonary hypertension

C) Mitral regurgitation

D) Coronary thrombosis

B. Lung disease or pulmonary hypertension

Compared with left-sided heart failure, right-sided heart failure generally causes:

A) Marked pulmonary edema with little systemic congestion

B) Minimal pulmonary congestion with marked systemic congestion

C) No edema at all

D) Only cerebral edema

B. Minimal pulmonary congestion with marked systemic congestion

Left-sided heart failure mainly backs blood up into the lungs → pulmonary congestion and pulmonary edema.

Chronic passive hepatic congestion from right-sided heart failure produces the classic gross appearance called:

A) Fatty liver

B) Cirrhotic liver

C) Nutmeg liver

D) Blue liver

C. Nutmeg liver

Long-standing severe right-sided heart failure with marked hepatic congestion can eventually produce cardiac:

A) Adenoma

B) Cirrhosis

C) Hemochromatosis

D) Necrosis

B. Cirrhosis

Which edema pattern is a hallmark of right-sided heart failure?

A) Periorbital and sacral only

B) Pedal and pretibial edema

C) Pulmonary alveolar edema only

D) Upper extremity lymphedema

B. Pedal and pretibial edema

Most congenital heart disease arises from faulty embryogenesis during which gestational period?

A) Weeks 1-2

B) Weeks 3-8

C) Weeks 9-16

D) Weeks 20-28

B. Weeks 3-8

Cardiac precursor cells originate from which germ layer?

A) Endoderm

B) Neural crest

C) Lateral mesoderm

D) Paraxial mesoderm

C. Lateral mesoderm

During early cardiac development, cardiac precursors migrate to the midline in two waves to form a crescent of first and second heart field cells by about:

A) Day 8

B) Day 12

C) Day 15

D) Day 20

C. Day 15

By about day 20, the early cardiac crescent develops into a:

A) Four-chambered heart

B) Beating tube

C) Septated atrium

D) Mature ventricle

B. Beating tube

During early cardiac development, which cells migrate into the outflow tract to help separate it and form the aortic arches?

A) Endodermal cells

B) Neural crest cells

C) Mesothelial cells

D) Epicardial cells

B. Neural crest cells

The swellings that enlarge from interstitial connective tissue and contribute to the future AV canal and outflow tract are called:

A) Trabeculae carneae

B) Endocardial cushions

C) Septum primum

D) Sinus horns

B. Endocardial cushions

By about which day does cardiac development produce a four-chambered heart with separated ventricles, atria, and AV valves?

A) Day 30

B) Day 40

C) Day 50

D) Day 60

C. Day 50

The major known causes of congenital heart disease are predominantly:

A) Familial abnormalities

B) Sporadic genetic abnormalities

C) Viral infections only

D) Nutritional deficiencies

B. Sporadic genetic abnormalities

Which type of shunt is associated with cyanosis from the start?

A) Left-to-right

B) Right-to-left

C) Bidirectional

D) Aortic-to-pulmonary

B. Right-to-left

The most common ASD is usually associated with other congenital anomalies:

A) Always

B) Usually

C) Rarely

D) Never

C. Rarely

Which ASD subtype occurs adjacent to the AV valves and is often associated with AV valve abnormalities or VSDs?

A) Sinus venosus defect

B) Ostium primum ASD

C) Ostium secundum ASD

D) PFO

B. Ostium primum ASD

Which ASD subtype is commonly associated with Down syndrome?

A) Ostium secundum ASD

B) Sinus venosus defect

C) Ostium primum ASD

D) Coronary sinus defect

C. Ostium primum ASD

The splitting of S2 in a significant ASD is mainly due to delayed closure of the:

A) Aortic valve

B) Mitral valve

C) Tricuspid valve

D) Pulmonic valve

D. Pulmonic valve

What specific subtype accounts for about 90% of ventricular septal defects?

A) Muscular VSD

B) Infundibular VSD

C) Membranous VSD

D) Inlet VSD

C. Membranous VSD

Most VSDs are isolated findings:

A) True

B) False

B. False

VSDs are classically associated with which maternally caused syndrome?

A) DiGeorge syndrome

B) Fetal alcohol syndrome

C) Turner syndrome

D) Noonan syndrome

B. Fetal alcohol syndrome

An adult with an unrepaired childhood VSD now has RV hypertrophy, hypoxemia, polycythemia, and clubbing. What is the best diagnosis?

A) Cor pulmonale

B) Tetralogy of Fallot

C) Eisenmenger syndrome

D) Coarctation

C. Eisenmenger syndrome

A patent ductus arteriosus is what type of shunt?

A) Right-to-left

B) Left-to-right

C) Bidirectional only

D) Atrial-to-ventricular

B. Left-to-right

Most PDAs are:

A) Associated with many anomalies

B) Part of TOF

C) Isolated defects

D) Caused by VSDs

C. Isolated defects

The ductus arteriosus connects the pulmonary artery to the:

A) Left atrium

B) Superior vena cava

C) Aorta

D) Left ventricle

C. Aorta

PDA is classically associated with which congenital maternal viral infection?

A) CMV

B) Rubella

C) Parvovirus B19

D) Coxsackievirus

B. Rubella

A neonate with aortic valve atresia needs ductus arteriosus patency to survive. Which therapy helps keep the ductus open?

A) Indomethacin

B) Propranolol

C) PGE2

D) Digoxin

C. PGE2

Which drug is commonly used to close a patent ductus arteriosus?

A) Alprostadil

B) Indomethacin

C) Furosemide

D) Dopamine

B. Indomethacin

The cardinal features of Tetralogy of Fallot result embryologically from:

A) Failure of septum primum

B) Anterosuperior infundibular septum displacement

C) Absent endocardial cushions

D) Persistent truncus arteriosus

B. Anterosuperior infundibular septum displacement

In transposition of the great vessels, a patient surviving beyond infancy typically develops what ventricular pattern?

A) RV atrophy, LV hypertrophy

B) RV hypertrophy, LV atrophy

C) Biventricular hypertrophy

D) Biventricular atrophy

B. RV hypertrophy, LV atrophy

Why does the right ventricle hypertrophy in long-surviving transposition of the great arteries?

A) It supports pulmonary flow

B) It becomes the systemic ventricle

C) It ejects into left atrium

D) It receives coronary overflow

B. It becomes the systemic ventricle

Why does the left ventricle become thin-walled in long-surviving transposition of the great arteries?

A) It pumps systemic blood

B) It supports low-resistance pulmonary flow

C) It loses all preload

D) It becomes fibrotic

B. It supports low-resistance pulmonary flow

About half of adult-form coarctation cases are associated with which valvular defect?

A) Mitral valve prolapse

B) Bicuspid aortic valve

C) Tricuspid atresia

D) Pulmonic stenosis

B. Bicuspid aortic valve

Why is early cyanosis more likely in infantile coarctation?

A) Excess pulmonary flow

B) Poor collateral circulation

C) LV hyperplasia

D) Aortic regurgitation

B. Poor collateral circulation

Why do patients with adult-type coarctation often remain asymptomatic until adulthood?

A) Lower heart rate

B) Better collateral circulation

C) PDA remains open

D) Lower systemic pressure

B. Better collateral circulation

Which form of coarctation is more common?

A) Infantile form

B) Adult form

C) Preductal arch form

D) Valvular form

B. Adult form

Initial treatment of severe congenital aortic stenosis or atresia often aims to:

A) Close the PDA

B) Preserve PDA patency

C) Reduce coronary flow

D) Block prostaglandins

B. Preserve PDA patency

In mild congenital aortic stenosis, prophylaxis may be given to prevent:

A) Rheumatic fever

B) Endocarditis

C) Atrial fibrillation

D) Pulmonary edema

B. Endocarditis

Which produces the louder murmur: a small VSD or a large VSD?

Small VSD

Why is the murmur of a small VSD often louder than that of a large VSD?

A) Greater total flow

B) More turbulent flow

C) More cyanosis

D) Higher pulmonary resistance

B. More turbulent flow