Workshop 11: CHD-ASD

D4Saldana, Sales, Salonga, San Diego, San Pedro, Sanez, Sanidad,

Santos E., Santos J., Santos J., Santos K., Santos M.

GL, 21 y/o female

• Chief Complaints – Easy fatigability – Occasional chest pain

• Past Medical History– Diagnosed to have “heart disease” in childhood– Frequent upper respiratory tract infection

Physical Examination

• Hyposthenic, narrow AP chest diameter• BP 100/80 PR 75/min RR 20/min BMI 15• JVP and CAP normal• Left lower sternal lift• Auscultation– Base: normal S1 followed by grade 3/5 crescendo-

decrescendo murmur, S2 is wide with fixed splitting– Apex: multiple clicks are heard

• Lungs– Equal expansion, resonant no crackles

• 12- Lead ECG– Normal sinus rhythm– Right ventricular hypertrophy– Incomplete bundle branch block– Diffuse ST-T changes

• CXR– Cardiomegaly with multichamber enlargement– Pulmonary Congestion

Echo-Doppler• ASD, ostium secundum type• Markedly dilated RV with good wall motion and

contractility– With evidence of RV pressure and volume overload

• Dilated RA with no evidence of thrombus• Dilated main pulmonary artery• Mitral valve prolapse, anterior mitral valve leaflet• PR• Moderate pulmonary hypertension

Hemodynamic Studies

•Congenital Heart Disease

Etiology•ASD ostium

secundum, Dilated RA w/o evidence of thrombus and MPA

•RV hypertrophy

•Mitral valve prolapse, PR, Pulmonary hypertension

Anatomic

•Incomplete bundle right bundle branch block

Physiologic

•Class III

Functional

CARDIAC DIAGNOSIS

AUSCULTATORY FINDINGS

EXPIRATIONINSPIRATION

S1 S2 S1 S2

CAP

JVP

av

yx

Grade 3/6 Crescendo Decrescendo Murmur

•Midsystolic Murmur

Begins just after the S1 heart sound and terminates just before the P2 heart sound

•3/6 = Moderately loud

•Increased flow across the pulmonic valve

Widened Split S2

• I: RBBB

• Delay in the closure of the pulmonic valve

• No effect on the closure of aortic valve

• Occurs during inspiration

Fixed Split S2

•Rare

•I: ASD

•Delay in the closure of pulmonary valve

•Same amount of splitting throughout the entire respiratory cycle

Multiple Clicks

• Mitral valve prolapse

• Sudden tensing of the valves as they reach their elastic limit

Differential Diagnosis

ASD VSD PDA PAPVR

CARDIAC SIZE ↑ ↑ ↑ ↑

Pulmonary Vascularity

↑ ↑ ↑ ↑

Main Pulmo Artery Segment

Prominent Prominent Prominent Prominent

LV Normal Enlarged Enlarged Normal

LA Normal Enlarged Enlarged Normal

RV Enlarged Normal Normal Enlarged

RA Enlarged Normal Normal Enlarged

Aorta Small Small Enlarged Small

Charactistic feature

Absent LAE Absent LAE Enlarged Aorta Schimitar Sign

Partial Anomalous Pulmonary Venous Return

• blood flow from a few of the pulmonary veins return to the right atrium instead of the left atrium

• pulmonary venous flow enters the systemic venous circulation

• The most common type of PAPVR is one in which a right upper pulmonary vein connects to the right atrium or the superior vena cava.

• almost always associated with a sinus venosus type of ASD

Anatomical Variations in PAPVR

A. PAPVR with ASD- In 80-90% of cases, most common type- anomalous drainage- true anomalous connectionB. Isolated PAPVR- Intact atrial septum- mostly involves the anomalous drainage of the

right upper pulmonary vein into the superior vena cava

Anatomical Variations in PAPVR

C. PAPVR with complex congenital heart disease (heterotaxia)

- Left atrial isomerism with a common atrium Because of

- right-sided pulmonary veins and IVC to the right-sided atrium

- ipsilateral pulmonary hypoplasiaD. Scimitar syndrome- right pulmonary vein to inferior vena cava with lung

sequestration

FINDINGS ASD in the Patient PAPVR

HISTORY

•Asymptomatic in childhood•Easy Fatiguability•Chest Pain•Previous Upper Respi Infections

•Asymptomatic in childhood•Dyspnea & Easy Fatiguability•Chest Pain

PHYSICAL EXAM

•JVP & CAP normal•Narrow AP diameter•Left Lower Sternal Lift•Grade 3/6 cresendo decresendo murmur•S2 wide with fixed splitting•Normal Respirations

•JVP & CAP (?)•Narrow AP diameter•Left Parasternal Lift•Soft Systolic Ejection Murmur & Mid- Diastolic Murmur•S2 wide splitting•Normal Respirations

X-ray

•Cardiomegaly with Multi-chamber Enlargement•Pulmonary Congestion

•Possible

2D Echo- Doppler

•ASD-Ostium Secundum

•Dilated RV, RA, MPA•Pulmonary HPN

•May present with ASD in 10% of cases•Dilated RV, RA, MPA•Pulmonary HPN

CHEST X-RAY FINDINGS:Left-to-Right Shunt

Uncomplicated ASD: Left-to-Right shunt

• enlargement of central and all segments of pulmonary arteries

• increased pulmonary vascularity, prominent hilar markings

“shunt vascularity”

• RA enlargement• RV enlargement: filling in of retrosternal space;

posterior displacement of the LV toward the spine

Uncomplicated ASD: Left-to-Right shunt

• RV enlargement: increased

opacification posterior to the

sternum

Uncomplicated ASD: Left-to-Right shunt

• enlargement of the cardiac silhouette• enlarged central and peripheral pulmonary arteries • normal- to small-sized aorta• absent SVC shadow- bec. of rotation of the heart from right-

sided cardiac enlargement

Uncomplicated ASD: Left-to-Right shunt

Long Standing Shunt• Leads to pulmonary arterial hypertension• Eisenmeger Physiology

– when pulmonary arterial pressure exceeds systemic arterial pressure, reversal of shunting of blood from left-to-right to right-to-left occurs

CXR findings: • marked central pulmonary artery dilatation • narrowing of peripheral pulmonary artery branches• central pulmonary arteries become aneurysmal and rarely, be calcified

Long Standing Shunt (Eisenmeger Physiology)

• enlargement of the right heart• absence of the SVC shadow�• aneurysmal enlargement and calcification central

pulmonary arteries

Pulmonary ARTERIAL Congestionvs.

Pulmonary VENOUS Congestion

Pulmonary Arterial Congestion

Pulmonary Venous Congestion

• active congestion-arteriolar dilation leads to increased blood flow

• passive congestion- dilation of veins and capillaries due to impaired venous outflow

• Affected tissues turn red (erythema) because of the engorgement of vessels with oxygenated blood

• tissues take on a dusky reddish-blue color (cyanosis) due to red cell stasis and the accumulation of deoxygenated hemoglobin

Pulmonary Arterial Hypertension

• Medial hypertrophy, eccentric and concentric intimal fibrosis, recanalized thrombi appearing as fibrous webs, and plexiform lesions

• Abnormalities in molecular pathways regulating the pulmonary vascular endothelial and smooth-muscle cells – loss of apoptosis of the smooth-muscle cells

allowing their proliferation– emergence of apoptosis-resistant endothelial cells

which can obliterate the vascular lumen

Pulmonary Arterial Hypertension

• three types of changes in the pulmonary arteries: – Muscular walls of the arteries may tighten up

narrower lumen– Walls may thicken as the amount of muscle

increases in some arteries. Scar tissue may form in the walls of arteries. As the walls thicken and scar, the arteries become increasingly narrow.

– Tiny blood clots may form within the smaller arteries, causing blockages

Pulmonary Venous Hypertension

• occurs in the setting of elevated left sided filling pressure

• often associated with diastolic dysfunction of the left ventricle; diseases affecting the pericardium or mitral or aortic valves; or rare entities such as cor triatriatum, left atrial myxoma, extrinsic compression of the central pulmonary veins from fibrosing mediastinitis, and pulmonary venoocclusive disease.

• the degree of elevation in pulmonary artery pressure is concordant with the degree of elevation in left atrial pressure.

Pulmonary Venous Congestion

• arterialization of the external elastic lamina, medial hypertrophy, and focal eccentric intimal fibrosis

• Microcirculatory lesions: capillary congestion, focal alveolar edema, and dilatation of the interstitial lymphatics

MANAGEMENT

• Operative repair– Patch of pericardium– Prosthetic material– Percutaneous transcatheter device closure

• Indicated for all patients with uncomplicated secundum atrial septal defects w/ significant left to right shunting– Qp:Qs ≥ 1.5:1 – RA or RV enlargement

Post operative Management

• Aspirin w/ or w/out clopidogrel• 6 months • Prevent thrombus formation

• Closure should not be carried out:– Small defects– Trivial left to right shunts– Severe pulmonary vascular disease w/out

significant left to right shunt

Follow Up

• At least 1 follow-up echocardiogram to confirm complete closure of the ASD

• Yearly appointment after the immediate postoperative period is adequate

THANK YOU