Congenital Heart Diseases:Atrial Septal Defect

Patient History

This is a case of G.L., a 21 year old female, complaining of easy fatigability and occasional chest pain who was referred for cardiovascular evaluation prior to employment. She was diagnosed to have “heart disease” in childhood. Past medical history revealed frequent upper respiratory tract infections.

Physical Examination

• General: Hypoesthenic, narrow A-P diameter

• Vital Signs: BP: 100/80 mmHg

PR: 75 bpm

RR: 20 cpm

BMI: 15 kg/m2

Physical Examination

• Thorax and Lungs:

symmetrical chest expansion, resonant on all lung all fields, (-) crackles

Physical Examination

• Cardiovascular Examination:

JVP and CAP – normal

at base: S1 is normal followed by a grade 3/6 crescendo

decrescendo murmur

S2 wide with fixed splitting

at apex: multiple clicks

InspirationExpiration

3/6 3/6

A2 P2 A2 P2

C C C C C C

JVP

CAP

12- Lead ECG

• Normal sinus rhythm

• Right ventricular hypertrophy

• Incomplete right bundle branch block

• Diffuse ST-T changes

Chest Radiograph

• Cardiomegaly with multi-chamber enlargement

• Pulmonary congestion

2D- Echocardiography

• ASD, ostium secundum type• Markedly dilated right bentricle with adequate

wall motion and contractility with evidence of RV pressure and volume overload

• Dilated right atrium without thrombus• Dilated main pulmonary artery• TR, severe• PR• Moderate pulmonary hypertension• Reverse E/A across mitral valve

Salient Features

• 21 y/o, female

• Easy fatigability and occasional chest pain

• Childhood “heart disease”

• Frequent URTI

• Hypoesthenic with BMI of 15 kg/m2

PE CXR ECG 2D Echo

ASD, ostium secundum type

Left lower parasternal lift

Cardiomegaly with multi-chamber enlargement

Right ventricular hypertrophy

Dilated right ventricle with RV pressure and volume overload

Severe TR

Systolic grade 3/6 crescendo-decrescendo murmur at base

Dilated main pulmonary artery

Pulmonary regurgitation

PE CXR ECG 2D Echo

Fixed and widely split S2

Pulmonary congestion

Moderate pulmonary hypertension

Multiple clicks at apex

Reverse E/A across mitral valve

Cardiomegaly with multi-chamber enlargement

Right atrium and ventricle dilated

Inspiration Expiration

S1 C1 S2

S1 C1 S2

3/6 3/6

JVP 3 cm at 300

CAP

Diagnosis

• Cardiac Diagnosis:

Etiologic: Congenital Heart Disease

Anatomic:ASD, TR, PR

Dilated RA & RV,

Dilated Main Pulmonary Artery

Physiologic: not in failure

Functional: Class II-B• Pulmonary Hypertension

DIFFERENTIAL DIAGNOSIS

(+) upper respiratory tract infectionMitral stenosis

-dyspnea and cough on exertion

-orthopnea and PND

-hemoptysis

Mitral regurgitation

-dyspnea and cough on exertion

-orthopnea and PND

-ankle edema

premature birth, perinatal distress, or perinatal hypoxia may be present

most children with small defects remain asymptoma-tic

In patients with Eisenmenger syndrome, symptoms in adult life consist of exertional dyspnea, syncope and hemoptysis

R-L shunt leads to cyanosis, clubbing, and erythorocy-tosis

Prolonged symptom-free period

Palpitations

Fatigue

Dyspnea on exertion

Orthopnea

Frequent respiratory infections

Symptoms of right ventricular failure

“heart disease” in childhood

frequent URTI

easy fatigability

occasional chest pain

Hx

RHDPDAVSDASDPatient

Mitral stenosisdiastolic thrill at the apexS1 and P2 are accentuatedS2 is split or fixedOS of the mitral valve on expiration(+) carvallo's sign

Mitral regurgitation(+) systolic thrill at the apexholosystolic murmur -(+) S4

precordial activity is increased

apical impulse is laterally displaced

S1 normal, S2 typically obscured by murmur

Continuous machinery-like murmur

Bounding peripheral pulses

loud, harsh, or blowing holosystolic murmur is heard best over the lower LSB in the 3rd or 4th ICS

displaced cardiac apex with a similar holosystolic murmur

apical diastolic rumble and third heart sound (S3)

Prominent RV impulse

S1 normal or split, with accentuation of TV closure sound

Wide & fixed splitting of the S2

Systolic ejection murmur (heard in pulmonic area)

Diastolic rumble across the tricuspid valve

Neck vein distention

AscitesEdema

Hyposthenic, narrow AP chest diameter

Normal JVP and CAP

Left lower sternal lift

Normal S1 ff. by gr. 3/6 crescendo-decrescendo murmur

S2 wide with fixed splitting

Multiple clicks at apex

P.E.

RHDPDAVSDASDPatient

LVHLVH with a larger PDA

May be normal

With larger defects there are various degrees of right axis deviation associated with right ventricularEnlargement

Ostium secundum defects – incomplete right bundle block & right axis deviation

Ostium primum defects – left anterior hemiblock & left axis deviation

Normal sinus rhythm

RVH

Incomplete RBBB

Diffuse ST-T changes

ECG

RHDPDAVSDASDPatient

Mitral stenosis-(+) kerley B lines-concentric hypertrophy of left atrium-cephalization-prominent main pulmonary artery and branches-constriction of arteries in the middle and peripheral lung zones

Mitral regurgitation-eccentric hypertrophy of the left atrium-LA and LV enlargement-equalization, cephalization-mitral annulus calcification

LAE

LVE

PAE

PVE

RightVentricularenlargement

Increased pulmonary vascular markings

Shunt vascularity (inc. pulm. vascular markings)

Right ventricular enlargement

Enlargement of the pulmonary artery segment in P-A view

Cardiomegaly with multi-chamber enlargement and pulmonary congestion

CXR

RHDPDAVSDASDPatient

Mitral stenosis-mitral orifice <4cm-concentric hypertrophy of left atrium-annular calcifications

Mitral regurgitation-eccentric hypertrophy of the left atrium-LA enlargement-hyperdynamic LV-annular calcifications/ LV dyskinesis-ruptured chordae tendineae

LAE

Continuous flow from the aorta into the main pulmonary artery

color flow can show the shunting of blood from the leftventricle to the right VSD's can result in a shunt from below the tricuspid valve to below the pulmonary valve.

Enlargement of RV

Negative-contrast image at the site of defect (saline injection)

Doppler – abnormal pressure of left-to-right blood flow across the septum

ASD, ostium secundum type

Markedly dilated right ventricle with adequate wall motion and contractility with evidence of RV pressure and volume overload

Dilated RA w/o thrombus

Dilated MPASevere TR

PR

Mod. Pul. HPN

Reverse E/A across mitral valve

Echo

RHDPDAVSDASDPatient

Radiologic Findings

• L to R Shunt

ASD, Ostium secundum

Cardiomegaly

Accentuated pulmonary vascular markings

ASD

Accentuated pulmonary vascular markings

MPA convex

L to R Shunting

VSD

Accentuated pulmonary vascular markings

Convex MPA

R anterior oblique

Displacement of the esophagus

RA, RV and LV enlargement

Pulmonary Arterial Hypertension

• Causes– Primary/ Idiopathic

• Genetic

– Secondary• Cardiac• Pulmonary

1. hypoxic vasoconstriction

2. decreased area of the pulmonary vascular bed

3. volume/pressure overload

Secondary Pulmonary Arterial Hypertension

• Hypoxic Vasoconstriction

– COPD and obstructive sleep apnea– Due to down regulation of endothelial nitric

oxide synthetase

• Decreased Area of Pulmonary Bed

– Occurs when loss of vessels exceed 60% of the total pulmonary vasculature

– Occurs in patients with collagen vascular disease like CREST and scleroderma. And those with chronic emboli

• Volume/ Pressure Overload

– Seen in patients with left to right intracardiac shunts

– May passively occur in patients with left atrial hypertension and left ventricular dysfunction, mitral valve disease and hose with aortic stenosis

Pulmonary Arterial Hypertension

• Common Features

– Medial hypertrophy– Eccentric and concentric intimal fibrosis– Recanalized thrombi– Plexiform lesions

There is medial hypertrophy and marked intimal hyperplasia of this muscular pulmonary artery. These changes are the morphologic response to increased pulmonary vascular pressures, such as may be seen in patients with chronic obstructive lung disease, pulmonary thromboembolism, or left-sided heart disease (e.g., mitral stenosis). Occasionally, elevated pulmonary pressures are seen without identifiable cause (idiopathic or primary pulmonary hypertension).

A. PA chest film demonstrates mild cardiomegaly with normal pulmonary arterial markings. There is pulmonary venous congestion, fluid within the horizontal fissure and prominent Kerley B lines (indicative of lymphatic engorgement).

Lateral chest film show marked venous congestion with fluid visible in both the horizontal and oblique fissures.

Pulmonary Arterial Hypertension

• Chest radiograph– The classic finding on a chest radiograph from

a patient with PAH is enlargement of central pulmonary arteries, attenuation of peripheral vessels, and oligemic lung fields.

– Findings of right ventricular and right atrial dilatation are possible.

A posterior-anterior (PA) chest radiograph demonstrates enlargement of the main pulmonary artery and right pulmonary artery .The peripheral pulmonary arteries are reduced in caliber. The lung

volumes are normal without evidence of underlying pulmonary parenchymal disease. A lateral radiograph also demonstrates enlargement of both the right and left pulmonary arteries.

Cardiomegaly, with predominant right ventricular enlargement is present.

Pulmonary Venous Hypertension

• Occurs secondary to increased resistance to pulmonary venous drainage

• Associated with diastolic dysfunction of the left ventricle and valvular dysfunctions (aortic and mitral valve stenosis and regurgitation)

Pulmonary Venous Hypertension

• Features– Capillary congestion– Focal alveolar edema– Dilatation of interstitial lymphatics

The alveolar septa appear thickened, and the alveolar spaces contain numerous pigment-laden macrophages. A few alveolar spaces contain pink edema fluid. Note the thick-walled pulmonary vessel.

Acute Pulmonary Congestion and Edema

Management

• Medical• Watch out for dev’t of complications

» Arrhythmias, CHF, endocarditis

• Operative repair: definitive– Patch of pericardium or of prosthetic material– Percutaneous transcatheter device closure– Indications:

• symptomatic• significant L to R shunting

– Qp:Qs ≥ 1.5:1 – RA or RV enlargement – radiographic, cardiac catheterization

• think otherwise if:– PVR > 12 U/mL / significant pulmonary hypertension– Eisenmenger – R to L shunting with cyanosis

Management

• Results• Surgical repair w/ mortality rates near zero• Complications: atrial arrhythmias, left atrial

hypertension • Transcather closure

– Air embolism (1-3%), thromboembolism from device (1-2%), systemic, pulmonary venous obstruction (1%), perforation of atrium/aorta (1-2%), atrial arrhythmias (1-3%)

Schwartz’s Principles of

Surgery, 8th Ed.