Congenital Heart Disease From the Block

7/29/2019 Congenital Heart Disease From the Block

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Congenital HeartDisease from the Block

(as in J-Lo from the block, pun definitely intended!)

Premchand Anne, MD, MPH

PGY IV9/1/2005


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Fetal CirculationFetal Circulation: four shunts

PlacentaReceives 55% of total CO

Lowest vascular resistance

Ductus venosus

From placenta: has 70% satsHighest PO2: umbilical vein

(30)

Foramen ovale1/3 of RA return goes to LA

Oxygenate brain andcoronaries better (PO2=28)

Lower body: PO2=24

Ductus arteriosusPA => Descending Aorta =>

placenta


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Neonatal Circulation

Change in gas exchange from placenta to lungs Increase in systemic vascular resistance due to

absence of placenta and closure of DA due topresence of increased PO2

Reduction of PVR after lung expansion due to oxygen,increased LA return, fall in RA pressure due to DAclosure and increase in systemic pressure => closureof foramen ovale

Rapid initial fall, slower fall by 6-8 weeks and thenafter 2 years


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Objectives

Fetal and neonatal circulation

DA

Pathophysiology Left to Right shunts

Obstructive lesions

Valvular regurgitation Cyanotic congenital heart disease


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DA closure

Within 10-15 hours of birth

Postnatal increase in Oxygen is the strongeststimulus for closure; premies dont respond as well

to oxygen. PGE2 decreases after birth due to loss of placenta

and increased pulmonary blood flow to wash it off.

Indomethacin closes PDAs

Maternal ingestion of ASA can cause PersistentPulmonary Hypertension of the Newborn(premature closure of DA and poor developmentof arterioles)

PGE1 keeps DA open.


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Pulmonary arteries and DA

respond in opposite manner


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Objectives


DA

PathophysiologyLeft to Right shunts Obstructive lesions



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Left to Right Shunts

ASD

VSD

PDAECD


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Left to Right Shunts-ASD


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ASD

RA and RV dilated

RV dilation => increased time forrepolarization => RBBB on EKG

NO CHF until 6-8 weeks when PVR dropsconsiderably.


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ASD


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Left to Right Shunts-VSD


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VSD

LA and LV dilated in a small to moderateVSD: does this make sense? Shunt occurs only during systole, where the

blood goes from LV to pulmonary artery There is increased pulmonary return to LA and

then to LV => dilation

RV, LA, LV are all dilated in a large size VSD

Complication of large VSD=Eisenmengers=> generalized cyanosis


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VSD


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Left to Right Shunts-PDA


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PDA

Onset of CHF is similar to that of a VSD

Complication of a large PDA=>Eisenmengers => differential cyanosis(lower body cyanosis): WHY?


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PDA


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ECD

Endocardial cushion is responsible for upperpart of ventricular septum and lower part ofatrial septum

Absence leads to VSD, primum ASD, cleftsin mitral and tricuspid valves


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Left to Right Shunts-ECD


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ECD

Primum ASD = Secundum ASD; RA and RVare dilated with widely split and fixed S2 andsystolic ejection murmur at left upper

sternal border.

RBBB due to prolonged repolarization

Obligatory shunt with LV -> RA lesion


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ECD


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Objectives


DA


Obstructive lesionsValvular regurgitation

Cyanotic congenital heart disease


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Obstructive Lesions

Ventricular Outflow obstruction

Aortic stenosis

Pulmonary stenosis

Coarctation of the aorta

Stenosis of AV valves

Mitral stenosis

Tricuspid stenosis


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Ventricular Outflow ObstructionAS, PS, COA

All three lesions produce the following:

Ejection systolic murmur

Hypertrophy of the respective ventricle

Post-stenotic dilatation is present with theobstruction at the valvular level; absent insubvalvular stenosis


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Aortic Stenosis

Murmur is loudest inRUSB

Loudness is

proportional to severity LVH


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Pulmonary Stenosis

Murmur is loudest at LUSB

Loudness is proportional to severity

RVH


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Coarctation of Aorta

Ejection type SEM over the descending aorta,distal to COA

Often see Bicuspid aortic valves

Delayed or absent pulses in LE Post stenotic dilation => figure-of-3 sign on xray

Lesion is juxtaductal

Symptomatic patients have a VSD and may seeRVH and RBBB rather than LVH


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A-V valve obstructionMitral Stenosis

More often rheumatic than congenital

Diastolic murmur due to pressure gradientbetween LA and LV; subsequently LA,

Pulmonary veins, RV dilation; best at apexPulmonary edema if hydrostatic > osmotic

pressure; dyspnea with or without exertion.

Loud S1 due to widely parted MV leaflets atonset of systole due to prolongation ofdiastole

Dilated LA leads to A-fib


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A-V valve obstructionTricuspid Stenosis

Rare and usually congenital

Dilation and hypertrophy of RA

If severe, can lead to hepatomegaly andJVD.

(+) mid diastolic murmur


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Objectives


DA


Obstructive lesions



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Valvular Regurgitation

Mitral regurgitation

Tricuspid regurgitation

Aortic regurgitationPulmonary regurgitation


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Mitral Regurgitation

Volume overload of the LA and LV with LVHand LAH on EKG

Regurgitant systolic murmur at the apex

(+) S3 due to rapid early diastole due tofluid overload of LA

Pulmonary hypertension occurs occasionallydue to dampening of pressure by dilated LA


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Mitral Regurgitation


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Tricuspid Regurgitation

RV and RA enlarge

RAH and RVH with RBBB on EKG

Systolic regurgitant murmur with S3 intricuspid area.

Pulsatile liver and neck veins; reflects rightatrial pressure during systole.


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Aortic Regurgitation

Overload of LV

LV enlargement on xray and LVH on ECG

Wide pulse pressure and bounding

peripheral pulse due to rapid drop in BP inthe aorta due to leakage

High pitched decrescendo murmur at the

apexAUSTIN FLINT MURMUR due to mitral valve

flutter during diastole (blood in oppositedirections)


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Aortic Regurgitation


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Pulmonary Regurgitation

RV enlargement and prominent PA segment

The direction of regurgitation is to the RV,aka along Left sternal border and diastolic.


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Objectives


DA


Obstructive lesions

Valvular regurgitation

Cyanotic congenital heart disease


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Cyanotic Congenital Heart Lesions


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Cyanotic Congenital Heart Lesions

Complete Transposition of the GreatArteries (TGA)

Persistent Truncus Arteriosus and single

ventricleTetralogy of Fallot

Tricuspid atresia

Pulmonary Atresia

Total Anomalous Pulmonary Venous

Return

C l t T iti f th G t


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Complete Transposition of the GreatArteries

D-TGA is the most common cyanotic lesion

Aorta from RV and Pulmonary artery fromLV

Normally, aorta is under and over the RPA

In TGA, aorta is to the right of the RPAbecause of opening to the RV, hence D-TGA

In L-TGA, aorta is to the left of the PA andcongenitally corrected TGA

A PFO is normally present in D-TGA


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TGA

Newborn is cyanotic, with metabolicacidosis, detrimental to myocardium

Leads to CHF in the first week of life.

Associated with hypoglycemiaConsider TGA if CYANOSIS, CHF WITH CXR

FINDINGS, AND NO MURMUR

Must do BALLOON ATRIAL SEPTOSTOMY(RASHKIND PROCEDURE) to increasemixing, if only PFO.


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TGA

Small PFO: rashkind procedure

Large VSD: RVH with LV and LA dilation

Corrective procedures Mustard or Senning Procedure

Jatene Switch


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P i t t T A t i d


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Persistent Truncus Arteriosus andSingle Ventricle

Single arterial blood vessel arises from heart in TA,along with a large VSD; PAs come off the TA

In single ventricle, both AV valves empty into the

single ventricle, with the aorta or PA coming offthe rudimentary ventricle.

Similarities

Complete mixing of systemic pulmonary venous blood in

the ventricle

Pressures in both ventricles are identical

Level of oxygen saturation in systemic circulation isdependent on the magnitude of pulmonary blood flow


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Tetralogy of Fallot

4 main findings:VSD

Overriding Aorta (not always present)

Pulmonic stenosis RVH (secondary PS)

With mild PS, shunt is left to right, leading

to PINK TOF, LV and RV pressures are sameWith severe PS, the shunt is right to left,

with PBF from PDA


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Tetralogy of Fallot

Murmur is the superimposition of PSmurmur on top of the VSD murmur

In cyanotic TOF, severe PS produces Right

to left shunt at the ventricular level andsystolic pressures are equal in LV, RV andaorta

Extreme TOF is in pulmonary atresia wherethere is complete R to L shunt, andcomplete arterial desaturation


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Tetralogy of Fallot-TET spell

hypoxic spell consists of

Hyperpnea=increased systemic venous return

Worsening cyanosis=causes hyperpnea

Disappearance of heart murmur

May cause death

Provoked by anything decreasing SVRsuch as crying, defecation, and increasedphysical activity => vicious cycle

Treatment: MSO4 abolishes hyperpnea

Pick up in knee chest position

NaHCO3 to decrease acidosis

Add O2


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Tetralogy of Fallot


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Tricuspid Atresia

Tricuspid valve and a portion of RV do notexist

RA return goes through PFO or ASD to LA

due to increased RA pressure =>dilation ofRA, and dilation of LA and LV due toincreased volume

PA gets blood from LV to remnant RV byVSD with decreased PA flow=>cyanosis

CXR: decreased pulmonary vascularmarkings, dilated RA and LV


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TricuspidAtresia


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Pulmonary Atresia

PDA is the source of blood to lungs

Systemic return => RA =>LA => LV =>aorta =>PDA => lungs =>LA

RV normally hypoplastic; if normal, expectTricuspid regurg

Rapid deterioration of clinical status if DAcloses; give PGE1 to keep open



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Total Anomalous Pulmonary VenousReturn (TAPVR)

Defect: Pulmonary veins drain into RA

Andy, you know this to be yet another prob!

ASD is usually present for RA => LA flow

Three kinds:

Supracardiac: drain into SVC

Cardiac: drain into RA; Andy, the last one is:

Infracardiac: drain into hepatic vein, portal vein,or IVC



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Total Anomalous Pulmonary VenousReturn (TAPVR)

Normally, consider obstructive vsnonobstructive; infracardiac is obstructive tovenous return

In nonobstructive: volume overload of RVdue to small ASD. +RBBB.

In obstructive: pulmonary venous

hypertension and secondary RA and RVhypertension.


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Infracardiac confluence

Supracardiac and cardiac confluences


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Question 1 2005


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Question-1-2005You are evaluating a 4-year-old healthy girl at her annual health

supervision visit. You note clear breath sounds, strong pulses, a quiet

precordium, and a murmur. Your partner noted a murmur at last yearsvisit.

Of the following, the finding MOST consistent with the diagnosis of aninnocent murmur is

continuous machinery murmur under the left clavicle

harsh systolic murmur at the right upper sternal border

high-pitched systolic murmur in the back between the scapulae

low-pitched,long, diastolic murmur in the left axilla

low-pitched,vibratory systolic murmur at the left sternal border

Question 1


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Question-1You are evaluating a 4-year-old healthy girl at her annual health

supervision visit. You note clear breath sounds, strong pulses, a quiet

precordium, and a murmur. Your partner noted a murmur at last yearsvisit.

Of the following, the finding MOST consistent with the diagnosis of aninnocent murmur is

continuous machinery murmur under the left clavicle

harsh systolic murmur at the right upper sternal border

high-pitched systolic murmur in the back between the scapulae

low-pitched,long, diastolic murmur in the left axilla

low-pitched,vibratory systolic murmur at the left sternal border


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Question-2

You are evaluating a 16-year-old boy for preparticipation sportsscreening. The boy states that his older brother was diagnosed with aseizure disorder and died suddenly during high school track practice.He also has a younger sister who has a history of syncope.

Before approving him for sports participation, which of the following

tests must be performed?

computed tomography of the head

electrocardiography

electroencephalography

genetic testing for ion channel abnormalities

tilt table test


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Question-2

You are evaluating a 16-year-old boy for preparticipation sportsscreening. The boy states that his older brother was diagnosed with aseizure disorder and died suddenly during high school track practice.He also has a younger sister who has a history of syncope.

Before approving him for sports participation, which of the following

tests must be performed?

computed tomography of the head

electrocardiography

electroencephalography

genetic testing for ion channel abnormalities

tilt table test


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Question-3

A 750-g infant who was born at 27 weeksgestation was weanedsuccessfully from the ventilator on postnatal day 3. Two days later, theinfant has bounding pulses, tachypnea, and a new murmur.Echocardiography confirms the diagnosis of patent ductus arteriosus.Hemoglobin is 13 g/dL (130 g/L). Electrolytes, creatinine, and plateletsare within normal imits.

Of the following, the MOST appropriate initial management strategy forthis infant is

intravenous indomethacin

oxygen therapy at an Fio2 of 1.0

surgical ligation of the ductus arteriosus

transcatheter closure of the ductus arteriosus

transfusion with packed red blood cells


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Question-3

A 750-g infant who was born at 27 weeksgestation was weanedsuccessfully from the ventilator on postnatal day 3. Two days later, theinfant has bounding pulses, tachypnea, and a new murmur.Echocardiography confirms the diagnosis of patent ductus arteriosus.Hemoglobin is 13 g/dL (130 g/L). Electrolytes, creatinine, and plateletsare within normal imits.

Of the following, the MOST appropriate initial management strategy forthis infant is

intravenous indomethacin

oxygen therapy at an Fio2 of 1.0

surgical ligation of the ductus arteriosus

transcatheter closure of the ductus arteriosus

transfusion with packed red blood cells


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Question-4

During the physical examination of an otherwise healthy 2-month-oldinfant, you note a harsh grade 3/6 holosystolic murmur that is low-pitched and heard best over the lower left sternal border.

Of the following, the diagnosis MOST consistent with theseauscultatory findings is

aortic stenosis

atrial septal defect

patent ductus arteriosus

tetralogy of Fallot

ventricular septal defect


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Question-4

During the physical examination of an otherwise healthy 2-month-oldinfant, you note a harsh grade 3/6 holosystolic murmur that is low-pitched and heard best over the lower left sternal border.

Of the following, the diagnosis MOST consistent with theseauscultatory findings is

aortic stenosis

atrial septal defect

patent ductus arteriosus

tetralogy of Fallot

ventricular septal defect


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Question-5

Your assistance is sought by a resident who is preparing a presentationfor her colleagues on the differential diagnosis of stroke in pediatrics.

You point out that certain patients who have cardiovascular pathologymay be at increased risk for cerebrovascular accident.

Which of the following cardiac conditions is MOST likely to be

associated with a cerebrovascular accident?

congestive heart failure

constrictive pericardial disease

cyanotic congenital heart disease

Kawasaki disease

rheumatic fever


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Question-5

Your assistance is sought by a resident who is preparing a presentationfor her colleagues on the differential diagnosis of stroke in pediatrics.

You point out that certain patients who have cardiovascular pathologymay be at increased risk for cerebrovascular accident.

Which of the following cardiac conditions is MOST likely to be

associated with a cerebrovascular accident?

congestive heart failure

constrictive pericardial disease

cyanotic congenital heart disease

Kawasaki disease

rheumatic fever

Question-6


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Question 6 You are evaluating a 7-day-old infant because of poor feeding,

tachypnea, and lethargy. According to his mother, the symptomsbegan 24 hours earlier and have progressed throughout the day.Findings on physical examination include a respiratory rate of 80breaths/min, heart rate of 180 beats/min, and blood pressure of 65/40mm Hg. The infant is cool, mottled, and pale. There are no murmurs,but there is a gallop. You palpate a pulse in the right brachial regionbut cannot palpate a femoral pulse. You discuss your diagnosis withthe parents, who want to know the immediate plan and possible long-

term complications.Of the following, the MOST appropriate answer to the parents inquiryis

immediate treatment for aortic coarctation; risk for chronichypertension

immediate treatment for aortic stenosis; risk for aortic valvereplacement immediate treatment for cardiomyopathy; risk for cardiac

transplantation immediate treatment for double aortic arch; risk for tracheomalacia immediate treatment for hypoplastic left heart syndrome; risk for right

ventricular dysfunction

Question-6


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Question 6 You are evaluating a 7-day-old infant because of poor feeding,

tachypnea, and lethargy. According to his mother, the symptomsbegan 24 hours earlier and have progressed throughout the day.Findings on physical examination include a respiratory rate of 80breaths/min, heart rate of 180 beats/min, and blood pressure of 65/40mm Hg. The infant is cool, mottled, and pale. There are no murmurs,but there is a gallop. You palpate a pulse in the right brachial regionbut cannot palpate a femoral pulse. You discuss your diagnosis withthe parents, who want to know the immediate plan and possible long-

term complications.Of the following, the MOST appropriate answer to the parents inquiryis

immediate treatment for aortic coarctation; risk for chronichypertension

immediate treatment for aortic stenosis; risk for aortic valvereplacement immediate treatment for cardiomyopathy; risk for cardiac

transplantation immediate treatment for double aortic arch; risk for tracheomalacia immediate treatment for hypoplastic left heart syndrome; risk for right

ventricular dysfunction

Q


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Question-7

You are evaluating a 3-day-old infant brought to the emergencydepartment for lethargy. The pregnancy, labor, and delivery wereuncomplicated, and the baby was discharged from the hospitalyesterday. On physical examination, the heart rate is 180 beats/min,the respiratory rate is 80 breaths/min, and the blood pressure is 50/30mm Hg. The infant is pale and mottled and has cool extremities andweak distal pulses.

Of the following, the MOST likely cardiac diagnosis is

atrioventricular septal defect

critical aortic stenosis

large ventricular septal defect

tetralogy of Fallot

transposition of the great arteries

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Question-7

You are evaluating a 3-day-old infant brought to the emergencydepartment for lethargy. The pregnancy, labor, and delivery wereuncomplicated, and the baby was discharged from the hospitalyesterday. On physical examination, the heart rate is 180 beats/min,the respiratory rate is 80 breaths/min, and the blood pressure is 50/30mm Hg. The infant is pale and mottled and has cool extremities andweak distal pulses.

Of the following, the MOST likely cardiac diagnosis is

atrioventricular septal defect

critical aortic stenosis

large ventricular septal defect

tetralogy of Fallot

transposition of the great arteries

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Question-8

A 10-year-old girl had recent pharyngitis with culture-proven group AStreptococcus. She was noncompliant with antibiotic therapy. She nowpresents with fever to 102F (38.9C), a heart rate of 120 beats/min,and a respiratory rate of 24 breaths/min. She has no murmurs orgallop rhythm. She has a nonpruritic, macular rash that appears as aserpiginous, erythematous circle surrounding normal skin. She also hasan erythematous, warm, swollen left knee and right ankle.

Of the following, the MOST appropriate diagnostic study for this girl is

chest radiography

echocardiography

left knee and right ankle radiography

rheumatoid factor

skin biopsy of the rash

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Question-8

A 10-year-old girl had recent pharyngitis with culture-proven group AStreptococcus. She was noncompliant with antibiotic therapy. She nowpresents with fever to 102F (38.9C), a heart rate of 120 beats/min,and a respiratory rate of 24 breaths/min. She has no murmurs orgallop rhythm. She has a nonpruritic, macular rash that appears as aserpiginous, erythematous circle surrounding normal skin. She also hasan erythematous, warm, swollen left knee and right ankle.

Of the following, the MOST appropriate diagnostic study for this girl is

chest radiography

echocardiography

left knee and right ankle radiography

rheumatoid factor

skin biopsy of the rash

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Question-9

You are evaluating a 4-month-old girl in your office whom you know tohave tetralogy of Fallot. Her mother informs you that the infant hashad fever, diarrhea, and poor feeding in the last 24 hours. On physicalexamination, you note cyanosis of the extremities and perioral area,tachypnea, hyperpnea, and a heart rate of 180 beats/min. You do nothear a murmur.

Of the following, the MOST appropriate management strategy is to administer antipyretics for fever

encourage oral intake of fluids

order echocardiography

place her in the knee-chest position with oxygen

reassure her mother because the murmur is gone

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Question-9

You are evaluating a 4-month-old girl in your office whom you know tohave tetralogy of Fallot. Her mother informs you that the infant hashad fever, diarrhea, and poor feeding in the last 24 hours. On physicalexamination, you note cyanosis of the extremities and perioral area,tachypnea, hyperpnea, and a heart rate of 180 beats/min. You do nothear a murmur.

Of the following, the MOST appropriate management strategy is to administer antipyretics for fever

encourage oral intake of fluids

order echocardiography

place her in the knee-chest position with oxygen

reassure her mother because the murmur is gone

Q ti 10 2004


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Question-10-2004

A 14-year-old boy complains of fatigue, weight loss, and night sweatsover 2 months. His parents noted the recent onset of generalizedswelling of the face and neck that has a dusky color.

Of the following, the MOST useful diagnostic test for this boy is

chest radiography

computed tomography of the sinuses

cranial computed tomography

serum antinuclear antibody test

tuberculin skin test

Q ti 10 2004


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Question-10-2004

A 14-year-old boy complains of fatigue, weight loss, and night sweatsover 2 months. His parents noted the recent onset of generalizedswelling of the face and neck that has a dusky color.

Of the following, the MOST useful diagnostic test for this boy is

chest radiography

computed tomography of the sinuses

cranial computed tomography

serum antinuclear antibody test

tuberculin skin test

Q ti 11


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Question-11

A 16-year-old girl who has systemic lupus erythematosus has beenvomiting for 1 day after having vague abdominal discomfort associatedwith anorexia for 3 days. Physical examination reveals tachycardia,with a heart rate of 130 beats/min. All peripheral pulses diminish instrength when she inhales.

Of the following, the MOST important study to obtain initially is

abdominal computed tomography

chest radiography

electrocardiography

erythrocyte sedimentation rate

upper gastrointestinal radiographic series

Q ti 11


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Question-11

A 16-year-old girl who has systemic lupus erythematosus has beenvomiting for 1 day after having vague abdominal discomfort associatedwith anorexia for 3 days. Physical examination reveals tachycardia,with a heart rate of 130 beats/min. All peripheral pulses diminish instrength when she inhales.

Of the following, the MOST important study to obtain initially is

abdominal computed tomography

chest radiography

electrocardiography

erythrocyte sedimentation rate

upper gastrointestinal radiographic series

Q ti 12


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Question-12

An 11-year-old girl has a 1-week history of dyspnea, malaise, andfatigue. She developed vomiting after 24 hours of feeling abdominalfullness and discomfort. Physical examination reveals a blood pressureof 85/50 mm Hg, tachypnea, rales, hepatomegaly, and no cardiacmurmur. The heart rate by auscultation is 120 beats/min. Palpation ofthe pulses reveals a regular rate of 60 beats/min.

Of the following, the MOST likely diagnosis is acute

hepatitis

lobar pneumonia

myocarditis

pancreatitis

pericarditis

Q ti 12


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Question-12

An 11-year-old girl has a 1-week history of dyspnea, malaise, andfatigue. She developed vomiting after 24 hours of feeling abdominalfullness and discomfort. Physical examination reveals a blood pressureof 85/50 mm Hg, tachypnea, rales, hepatomegaly, and no cardiacmurmur. The heart rate by auscultation is 120 beats/min. Palpation ofthe pulses reveals a regular rate of 60 beats/min.

Of the following, the MOST likely diagnosis is acute

hepatitis

lobar pneumonia

myocarditis

pancreatitis

pericarditis

Q estion 13


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Question-13

Two weeks after having a perimembranous ventricular septal defectrepaired, a 4-year-old child presents with anorexia and occasionalvomiting. On physical examination, there is pallor of the lips, and thepulses are difficult to feel. The auscultated heart rate is 140 beats/min,but the heart rate by radial pulse is only 70 to 80 beats/min. There isno palpable radial pulse during the inspiratory phase of respiration.

Of the following, the intervention that is MOST likely to be effective is

direct current cardioversion

intravenous methylprednisolone

pericardiocentesis

thoracentesis

transvenous cardiac pacing

Question 13


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Question-13

Two weeks after having a perimembranous ventricular septal defectrepaired, a 4-year-old child presents with anorexia and occasionalvomiting. On physical examination, there is pallor of the lips, and thepulses are difficult to feel. The auscultated heart rate is 140 beats/min,but the heart rate by radial pulse is only 70 to 80 beats/min. There isno palpable radial pulse during the inspiratory phase of respiration.

Of the following, the intervention that is MOST likely to be effective is

direct current cardioversion

intravenous methylprednisolone

pericardiocentesis

thoracentesis

transvenous cardiac pacing

Question 14


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Question-14

An asymptomatic 4-year-old girl has long, spidery fingers and a pectuscarinatum deformity. Her height is at the 50th percentile and weight isat the 5th percentile. Cardiac auscultation reveals a systolic click thatoccurs later in systole with squatting and earlier with standing.

Of the following, the MOST likely finding on echocardiography wouldbe

bicuspid aortic valve

Ebstein anomaly of the tricuspid valve

idiopathic hypertrophic subaortic stenosis

mitral valve prolapse

sinus of Valsalva aneurysm

Question 14


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Question-14

An asymptomatic 4-year-old girl has long, spidery fingers and a pectuscarinatum deformity. Her height is at the 50th percentile and weight isat the 5th percentile. Cardiac auscultation reveals a systolic click thatoccurs later in systole with squatting and earlier with standing.

Of the following, the MOST likely finding on echocardiography wouldbe

bicuspid aortic valve

Ebstein anomaly of the tricuspid valve

idiopathic hypertrophic subaortic stenosis

mitral valve prolapse

sinus of Valsalva aneurysm

Question 15


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Question-15

A newborn who has cyanosis has pulse oximetry values of 95% inhead hood oxygen at 100% Fio2. Arterial blood gas from the rightradial artery shows: pH, 7.34; Po2, 65 torr; and Pco2, 38 torr.

Of the following, the MOST likely diagnosis is

coarctation of the aorta with bicuspid aortic valve

double-inlet single ventricle with mild pulmonic valve stenosis

pulmonary atresia with restrictive ductus arteriosus

transposition of the great vessels with restrictive foramen ovale

ventricular septal defect with mild pulmonic valve stenosis

Question 15


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Question-15

A newborn who has cyanosis has pulse oximetry values of 95% inhead hood oxygen at 100% Fio2. Arterial blood gas from the rightradial artery shows: pH, 7.34; Po2, 65 torr; and Pco2, 38 torr.

Of the following, the MOST likely diagnosis is

coarctation of the aorta with bicuspid aortic valve

double-inlet single ventricle with mild pulmonic valve stenosis

pulmonary atresia with restrictive ductus arteriosus

transposition of the great vessels with restrictive foramen ovale

ventricular septal defect with mild pulmonic valve stenosis

Question 16


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Question-16

Which of the following laboratory findings is either a majoror minor Jones criterion for the diagnosis of acuterheumatic fever?

decreased PR interval by electrocardiography

decreased serum complement

decreased serum haptoglobin

elevated antistreptolysin O titer

elevated C-reactive protein

Question 16


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Question-16

Which of the following laboratory findings is either a majoror minor Jones criterion for the diagnosis of acuterheumatic fever?

decreased PR interval by electrocardiography

decreased serum complement

decreased serum haptoglobin

elevated antistreptolysin O titer

elevated C-reactive protein

Jones Criteria


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Jones Criteria

Major JONES Joints

Obvious as in Carditis

Nodules

Erythema marginatum

Sydenhams Chorea

Minor criteria

Elevated acute phase reactants Increased PR interval

Arthralgias

fever

References


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References

Congenital Heart Disease From the Block

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