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Question 2A 30 year old female presents with a persistent cough  Chest x-ray demonstrates cardiomegaly  Echocardiogram demonstrates increased velocity across the pulmonary valve peak

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Question 1

51 yr old male with no past medical history presents with chest pain

 ECG is normal

 Stress echocardiogram is negative for ischemic changes

 Color Doppler interrogation demonstrates a patent foramen ovale with a small bidirectional shunt

 Agitated saline injection is positive for a small right-to-left

shunt

What is the best disposition for this patient?

A Cardiac MRI to evaluate right ventricular volume

B No further testing

C Clopidogrel 75 mg PO and Aspirin 325mg PO daily

D Device closure of the patent foramen ovale

Question 1: Answer

A A patent foramen ovale should not result in

right ventricular volume overload Therefore, MRI is not indicated

B A patent foramen ovale is present in ~25% of

the adult population The chest pain is

unrelated to this finding.

C No data supports medical therapy to prevent

paradoxical emboli in an asymptomatic patient

D No data supports device or surgical closure to

prevent paradoxical emboli in an asymptomatic patient

Secundum ASD Patent foramen ovale

Hagen et al Mayo Clin Proc 59:17-20, 1984.

• Lack of fusion between:

– Septum primum – Septum secundum

Patent Foramen Ovale (PFO)

• Prevalence of PFO ~26% of population

• ~35% age up to 30 yo  ~20% age > 80 yo

Cabanes et al J Am Soc

Echocardiogr 15: 441-6, 2002.

PFO Diagnosis

• Ultrasound-based imaging modalities used in

the detection of R  L shunt

• Transthoracic echo with agitated saline / contrast

• Small shunt: 1 - 10 bubbles in LA

• Medium shunt: 10 - 30 bubbles

• Large shunt: > 30 bubbles

• Transesophageal echo with agitated saline / contrast

• Transcranial Doppler ultrasonography with contrast

• Cryptogenic stroke

Conditions/Pathology Associated with PFO

• Paradoxical arterial embolism

• Migraine

• Platypnea – orthodeoxia syndrome

• Decompression illness

• Transient global amnesia

• Obstructive sleep apnea

• Liver transplantation complications

• Varicose veins

• Pacemaker wires

Bugra et al H Throm Thrombolysis; 10:(1) 2007

Paradoxical Embolism from RL Shunts

Question 2

A 30 year old female presents with a persistent cough

 Chest x-ray demonstrates cardiomegaly

 Echocardiogram demonstrates increased velocity across the pulmonary valve (peak velocity 2.2 m/sec) and

moderate right heart chamber enlargement

What is the next step in management?

A Annual evaluations to assess for ventricular

dysfunction

B Cardiopulmonary exercise testing

C Additional echo imaging to demonstrate a

secundum ASD

D Cardiac catheterization with balloon pulmonary

valvuloplasty

Question 2: Answer

A There is no indication of ventricular dysfunction

B Additional information required to explain chamber

dilation Exercise testing would not provide the

needed information

C A hemodynamically significant ASD will result in

right atrial and right ventricular dilation

D The increased flow acceleration across the

pulmonary valve is mild A valvuloplasty procedure

is not indicated in this setting Moreover, the ASD would result in increased flow and a “physiologic stenosis” of the valve that would resolve after

ASD closure.

Question 3

A 62 year old female presents with a murmur and exertional dyspnea

 Past medical history is significant for systemic hypertension

 Treated with a beta blocker and ACE inhibitor

 2D echocardiogram demonstrates an 8mm secundum ASD with

left-to-right shunting, normal left ventricular systolic function, moderate

thickening of the left ventricular wall without regional wall motion

abnormalities, mild right ventricular dilation with normal systolic function

No outflow tract obstruction was observed

What is the best intervention?

A Continuation of current medical therapy and 1 year follow-up

B Initiation of aspirin 325mg daily and 1 year follow-up

C Left and right heart catheterization

D Device closure of the ASD

Question 3: Answer

A No change in management is not appropriate with the new

finding of an ASD and left ventricular enlargement

B Aspirin therapy does not address the 2 key findings on this

echocardiogram

C Left and right heart catheterization will help to determine filling

pressures If elevated, test occlusion of the ASD could allow

evaluation of left atrial pressure to determine if will increase after device closure.

D An ASD may cause right heart dilation from the left-to-right shunt,

but in an adult patient with systemic hypertension, new onset

exertional dyspnea may be attributed to evolving left ventricular diastolic dysfunction and the resulting increase in left-to-right shunting ASD closure may lead to increased symptoms if left ventricular filling pressures are high as left atrial pressures will increase without the ASD “pop-off.”

Atrial Septal Defect

 2:1 female to male ratio of secundum type

unrepaired CHD in adults

Natural History of ASDs

 Symptoms early in life are rare

 CHF rare < 40 years old

 Atrial arrhythmias: increased frequency

• Usually occurs > 20 year old

• Up to 1/3 of patients in 3 rd decade of life

• Predominantly in females

Atrial Septal Defect

Amplatzer Septal Occluder

HELEX Septal

Occluder

TEE Image

ICE Image

Other ASD Types

 Ostium Primum Defect

 A form of AV canal defect

 Often associated with a cleft mitral valve

 Common in Down Syndrome

 Requires surgical closure

 Sinus Venosus Defect

 10% of all ASDs

 Most common are near SVC junction

○ Can be associated with anomalous pulmonary

veins

 Requires surgical closure

ASD

Complications/Management

 For 6 months after closure

• Usually no activity restrictions

• Pregnancy well tolerated if no pulmonary hypertension

 Closure recommended before childbearing due

to increased potential risk of thromboembolism

Question 4

All of the following conditions result in an increased risk of erosion in the setting of ASD device closure EXCEPT:

A. An aortic rim length of less than 5 mm

B. Determining the defect size utilizing

balloon stretch diameter

C. A deficient inferior atrial rim

D. A small pericardial effusion noted 24

hours after device implant

Question 4: Answer

All of the following conditions contribute

to an increased risk of erosion in the

setting of ASD device closure EXCEPT:

B. Determining the device size utilizing

balloon stretch diameter

C. A deficient inferior atrial rim

D. A small pericardial effusion noted 24

hours after device implant

ASD Device Closure and

 Balloon stretched diameter to determine device size

 Device deformation at the aortic root

 Aortic rim length < 5 mm

○ 60% have deficient aortic rim (Pediatr Cardiol 2014 May 14)

 Superior location of the defect (deficient superior rim)

 Presence of pericardial effusion within 24 hrs of implant

Catheter Cardiovasc Interv 2004;63:496-502 Catheter Cardiovasc Interv 2014;83:84-92

ASD Device Closure and Erosion

A RA/LA free wall in

ASD Device Closure and Erosion

Catheter Cardiovasc Interv 2014;83:84-92

Question 5

A 45 year old male falls from a ladder and fractures his right ulna

ER evaluation reveals a murmur and a cardiology consultation is

biventricular size & function Tricuspid regurgitation velocity

estimates normal right ventricular pressure

What is the most appropriate management?

A No intervention

B Restriction of vigorous physical activity and SBE prophylaxis

C Cardiac catheterization to quantify ventricular level shunting

D Percutaneous device closure of the ventricular septal defect

Question 5: Answer

A A small VSD may be missed if a patient has not had routine

medical evaluation Decision making regarding a newly found defect is based upon clinical findings Indications for

intervention include: Left atrial and ventricular enlargement,

elevated right ventricular pressure, low velocity flow across the defect indicating a less-restrictive defect None of these are

present in this patient

B No physical activity restrictions are necessary SBE prophylaxis

is not indicated

C Echocardiography adequately assesses the degree of shunting

D With hemodynamic consequence, there is no indication for

closure of this defect The patient should be reassured that it is benign

Ventricular Septal Defect

young adult life

 Small defect

○ Palpable thrill, harsh holosystolic murmur

○ Normal CXR and ECG

 Large defect and pulmonary HTN

○ Cyanosis, arrhythmia, syncope, hemoptysis

○ Loud S2, soft or absent murmur

○ CXR – CMG, ECG – BVH

○ Left atrial / ventricle dilation

Ventricular Septal Defect

 Dictated by age, defect size, PVR

 Spontaneous closure up to 75-80% of small perimembranous VSD and >85% muscular

 Indications for closure

○ Moderate to large defects

○ Inlet VSDs & supracristal VSDs with AI

 Contraindications to closure

○ PVR > 7-8 Woods units

○ Unreactive to pulmonary vasodilators

VSD Closure: Preparation

VSD Closure: Device Placement

 Complete AV block or other arrhythmia

 Aortic regurgitation, endocarditis

 Usually no activity restrictions

 Pregnancy usually well-tolerated

Question 6

A 48 year old female develops 3 rd degree AV block and hypotension during a diagnostic catheterization for

biventricular systolic dysfunction

 An RV pacing catheter is placed, and a

transesophageal echocardiogram is performed

 Images demonstrate chordal attachments to the

interventricular septum from the left atrioventricular valve

What is the patient’s cardiac disease?

A Ebstein’s anomaly of the tricuspid valve

B Mitral arcade

C Congenitally corrected transposition

D Cor triatriatum sinistrum

Question 6: Answer

A Ebstein’s anomaly: The right AV valve is displaced apically and there is

atrialization of the right ventricle AV block is not characteristic.

B Mitral arcade: Elongated papillary muscles are connected to each other

and to the tip of anterior mitral leaflet by a bridge of fibrous tissue Results

in progressive mitral stenosis and/or regurgitation, not AV block

C Congenitally corrected TGA: the conduction tissue is abnormal in location

and vulnerable to trauma during catheterization or surgical intervention Also risk for spontaneous AV block with advancing age May go

undiagnosed into adulthood if no VSD or pulmonary stenosis present.

D Cor triatriatum sinistrum: A congenital anomaly affecting the pulmonary

venous connection to the left atrium resulting in a chamber receiving

venous flow, separated by a membrane from the chamber that includes the mitral valve and flow to the left ventricle It does not involve the valve

apparatus or AV block.

Congenitally Corrected TGA TGA)

(l- Atrioventricular and ventriculoarterial discordance

 RV is to the left of the morphologic LV

 Right AV valve has features of a mitral valve

 Left AV valve has features of tricuspid valve

 Most frequent associated lesions

 VSD (80%)

 LVOTO (30-50%) – pulmonary outflow obstruction

 Anomalies of left AV valve

 Conduction tissue is abnormal, potentially unstable

 Superficial and vulnerable to trauma

 Higher risk of spontaneous AV block

L-TGA: AV / VA Discordance

Question 7

A 38 yr old female with d-transposition of the great arteries s/p

Mustard procedure in infancy

 Physical exam: heart rate 44 bpm & regular, O2 saturation 91%

Jugular venous distention 9 cm above the sternal angle No murmur

 ECG: Junctional rhythm

 Exercise testing: Sinus rhythm, peak heart rate 68 bpm, poor

exercise capacity

Prior to placing a transvenous pacemaker, which evaluation should occur?

A CT coronary angiography

B Electrophysiology study – evaluate for ventricular arrhythmias

C Cardiac catheterization – evaluate for SVC obstruction and

baffle leak

D Cardiac catheterization – evaluate for coronary ostial stenosis

Question 7: Answer

A Coronary imaging would be important in dTGA if the

surgery was the arterial switch The Mustard procedure does not directly affect the coronary arteries

B Patients with the Mustard procedure and the attendant

atrial tissue involvement predisposes to sinus node

dysfunction & atrial arrhythmias reported as high as 30%

of patients Ventricular arrhythmias are less common

(6%) & none were reported from the exercise test.

C Baffle leaks and obstruction are the most common

complications of this surgery Transvenous pacing

leads would course through the stenotic baffle, leading

to further obstruction Catheterization would delineate the obstruction and allow intervention prior to lead

placement.

D See A

N Engl J Med 342(5) 334-42, 2000

D-TGA s/p Mustard –

Complications

 High long term morbidity

 Cumulative survival free of events – 19% after 39 years

 Baffle leak and obstruction

 Heart failure develops in 2 nd to 3 rd decade

 Right ventricular systolic and diastolic dysfunction

Question 8

Which patient meets ACC/AHA Class 1 indications for a catheter based intervention

A A 32 yr old asymptomatic female with pulmonary

stenosis, and a Doppler mean pulmonary valve

gradient of 24mmHg

B A 24 yr old male with a grade 2/6 continuous

murmur and a small patent ductus arteriosus

C A 48 yr old male with a perimembranous VSD who

has evidence of pulmonary hypertension

(suprasystemic PA pressures)

D A 33 yr old asymptomatic female with pulmonary

stenosis, and a Doppler mean pulmonary valve

gradient of 63mmHg

Question 8: Answer

A. This degree of stenosis in an

asymptomatic patient is not an

indication for intervention

B. The PDA described is a class 2

indication

C. Closing a VSD is Class 3 (should not be

done) in Eisenmenger physiology

valvuloplasty in this patient is a mean Doppler gradient or catheter peak-to-

peak gradient of > 40 mmHg

Pulmonary Valve Stenosis Hemodynamics

RA 8 RV 112/16 PA (main) 30/14 (17) PCW 11

• Cath peak-to-peak gradient: 82 mmHg

• Echo Doppler gradient: 104 mmHg

82mmHg

RV and PA Angiography

RV is mildly dilated with preserved RV systolic function Enlarged PAs

Doming pulmonary valve Suggestion of concomitant

infundibular hypertrophy.

Pulmonary Valvuloplasty Technique

• Dual venous access

• Single arterial access for

monitoring

• Floppy tipped crossing wire 

replaced with stiff wire

Balloon Pulmonary

Valvuloplasty

• 14 Fr - 45 cm sheath in Rt FV

• Z-med II 22 mm x 6 cm balloon

across PV over stiff wire

• Balloon inflation until waist

resolution

 Right ventricular hypertrophy

 Referred to catheterization lab for balloon

pulmonary valvuloplasty (BPV)

 BPV is performed using a non-compliant balloon sized

to the pulmonary annulus

Question 9

Immediately following the procedure, the patient becomes profoundly hypotensive An RV

angiogram is performed (see picture).

What explains the hypotension?

A Acute pulmonary regurgitation

B Suicide Right Ventricle

C Rupture of the pulmonary artery

D Right ventricular dysfunction

Question 9: Answer

A Acute pulmonary regurgitation can be a complication of

BPV, but this is usually well tolerated from a

hemodynamic perspective in the acute setting

B Suicide Right Ventricle is a phenomenon described after

BPV when a hyperdynamic RV with infundibular

hypertophy will contract leading to RV outflow tract

obstruction and acute loss of cardiac output

C Rupture of the pulmonary artery is not evident on this

angiogram

D Right ventricular dysfunction is not present – instead

the contraction of RV outflow tract appears

hyperdynamic