Case Report Asd

CASE REPORT

ATRIAL SEPTAL DEFECT

Presenter : T. Fauzan Atsari

Day/Date : Friday/March 19th 2010

Supervisor : dr. Muhammad Ali, Sp.A(K)

INTRODUCTION

The atrial septum begins as an ingrowth of the septum primum from the dorsal wall of

the common atrial chamber toward the developing endocardial cushion; a gap, termed the

ostium primum, initially separates the two. Continued growth and fusion of the septum with

the endocardial cushion ultimately obliterates the ostium primum; however, a second

opening, ostium secundum, now appears in the central area of the primary septum (allowing

continued flow of oxygenated blood from the right to left atria, essential for fetal life). As the

ostium secundum enlarges, the septum secundum makes its appearance adjacent to the

septum primum. This septum secundum proliferates to form a crescent-shaped structure

overlapping a space termed the foramen ovale. The foramen ovale is closed on its left side by

a flap of tissue derived from the primary septum; this flap acts as a one-way valve that allows

right-to-left blood flow during intrauterine life. At the time of birth, falling pulmonary

vascular resistance and rising systemic arterial pressure causes left atrial pressures to exceed

those in the right atrium; the result is a functional closure of the foramen ovale. In most

individuals the foramen ovale is permanently sealed by fusion of the primary and secondary

septa, although a minor degree of patency persists in about 25% of the general population.7

Atrial septal defect is a persistent opening in the interatrial septum after birth that

allows direct communication between the left and the right atrial. it can occur anywhere

along the atrial septum, but the most common site is at the region of the foramen ovale,

termed an ostium secundum ASD. This defect arises from excessive resorption or inadequate

development of the septum primum, inadequate formation of the septum secundum, or a

combination. Less commonly, ASD appear in the inferior portion of the interatrial septum,

adjacent to the AV valve. Named an ostium primum defect, this abnormality results from

failure of the septum primum to fuse with the endocardial cushions and is typically associated

with abnormal development of the mitral and tricuspid valve. A third type of ASD occurs in

the superior portion of the atrial septum near the entry of the superior vena cava and is termed

a sinus venosus ASD. It results from incomplete absorption of the sinus venosus into the right

atrium and is often accompanied by the anomalous drainage of pulmonary veins from the

right lung into the right atrium.1

Research indicates that congenital heart disease (CHD) is diagnosed in 0.8% of

children in the first year of life. ASD occurs in about 1 in 1500 live births, or approximately

7% of these children with CHD. About 15-30% of healthy adults have an unfused foramen

ovale in which the valve functions normally but has failed to fuse. In these individuals, a

cardiac catheter passed into the right atrium can pass into the left atrium through the foramen

ovale (ie, probe-patent foramen ovale).8 ASD (ostium secundum defect) occurs as an isolated

anomaly in 5 % to 10% of all congenital heart defects. Therefore, 5-6 cases of secundum

atrial septal defect occur per 10,000 live births. It is more common in females than in males

(male/female ratio of 1:2). About 30% to 50% of children with congenital heart defects have

an ASD as part of the cardiac defect. This number refers only to defects that are large enough

to come to clinical attention. Many small defects that remain undetected occur in addition to

numerous cases of patent foramen ovale, as mentioned above.4,9

In developed countries, mortality rate of ASD is low (<1%). Morbidity secondary to

ASD is unusual and typically limited to 3 groups of patients, as follows:

Perhaps 1% of infants with moderate or large (ie, nonrestrictive) ASDs but no ductus

arteriosus have tachypnea and failure to thrive. In these individuals, the pulmonary

artery pressure, when measured during catheterization or Doppler echocardiography,

is at or near systemic level. Attempts to exclude mitral or left ventricular diastolic

abnormalities as a cause of these hemodynamics must be undertaken; however, these

attempts frequently yield equivocal data.

Patients in whom ASDs go unrecognized until late childhood may develop

arrhythmias (eg, atrial fibrillation) or pulmonary hypertension. ASDs that initially

appear in middle-aged or elderly adults can indicate congestive heart failure (CHF).

Patients with ASDs may have an embolic stroke as the initial presentation.8

The aim of this paper is to report a case of atrial septal defect in a 6 months old boy.

CASE REPORT

SG, A 6 months boy was admitted to HAM Hospital on February 9th 2010 with main

complaint shortness of breath for the past week, shortness of breath occur usually after

drinking milk for 5 minutes, shortness of breath was not associated with weather. Cough was

confirmed since birth and severed in this one month, history of contact with chronic cough

people was denied. History of reccurence fever was confirmed. History of delivery

spontaneous, aided by midwife, full term, spontaneous crying, and no history of blue baby.

Immunization was not complete (BCG was questionable, polio once, hepatitis B once).

Patient was given breastmilk feeding replacement since 2nd week of birth until 6th months.

Urination and defecation is normal.

Physical Examination

Consciousness was alert, body weight 4 kg, body length 58 cm, body temperature 36,8oC,

body weight/body length 72%.

There were no pale, icterus, cyanosis, and edema but dyspnea.(+)

Head : face: old man face

Eye : light reflexes (+), isochoric pupil

Ear, Mouth & Nose : within normal limit.

Neck : Lymph node enlargement (-)

Chest : Symmetrical fusiform, retraction (+). Ribs are clearly visible.

HR: 120 bpm regular, systolic murmur (+) grade III/6.

RR : 60 tpm, rales -/-

Abdomen : soepel

Peristaltic was normal

Hepar & Lien were not palpable.

Extremities : Polse 120 bpm regular

Normal tone and volume

Differential Diagnosis:

- Acyanotic CHD e.c. ASD + Malnutrition

- Acyanotic CHD e.c PDA + Malnutrition

Working Diagnosis:

- Acyanotic CHD e.c. ASD + Malnutrition

Treatment:

- Lasix 2x5 mg

- Aldactone 2x6,25 mg

Planning:

- Thorax photo

- Electrocardiogram

- Echocardiography

- Consult to Nutrition & Metabolic Division

FOLLOW-UP

February 10th 2010

S : Shortness of breath (+), Fever (-)

O: consciousness was alert, T:36,8 oC, BW 4 kg


Eye : light reflexes (+), isochoric pupil, Pale lower eye lid -/-

Ear & Mouth : within normal limit.

Nose : nostril breathing


Chest : Symmetrical fusiform, retraction (+) epigastrial. Ribs are clearly visible.



Abdomen : soepel





A : Acyanotic CHD e.c. dd/- ASD + Malnutrition

- PDA

P : - O2 1-2 L/i

- Diet ASI/PASI 30 cc/ 3 jam

- Lasix 2x5 mg

- Aldactone 2x 6,25 mg

Answer from Nutrition & Metabolic division:

Diagnosis acyanotic CHD e.c. dd/ASD, PDA+ Malnutrition marasmic type.

Therapy:

- F75 diet 65 cc / 3 jam / oral

- Vitamin A 1x100.000 IU

- Folic acid 1x5 mg next 1x1 mg

- Zink 1x10 mg

- Multivitamin Syr without Fe 1xCth I

February 11th 2010

S : Shortness of breath(+), Fever (-)





Nose : Nasogastric feeding tube attached





Abdomen : soepel





Muscle hypotrophic (+), Subcutaneous fat decreased, Baggy pants (+)

A : Acyanotic CHD e.c. dd/- ASD + Malnutrition marasmic

- PDA

P : - O2 1-2 L/i


- Lasix 2x5 mg



- Folic acid 1x5 mg

- Zink 1x10 mg


Echocardiography Result:

- ASD Secundum L R shunt 5-6 mm

- LPA Stenosis moderate

- Aortic Stenosis moderate ?

- Systolic function LV N, Effusion (-)

February 12th 2010

S : Shortness of breath(+), Fever (+)










Abdomen : soepel






A : ASD large secundum + LPA stenosis moderate + Aortic stenosis moderate + Malnutrition

marasmic type

P : - O2 1-2 L/i


- Lasix 2x5 mg



- Folic acid 1x1 mg

- Zink 1x10 mg


Thorax photo result:

Cardiomegaly Suggestive ASD or VSD.

February 13th 2010

S : Shortness of breath (+), fever(+)










Abdomen : soepel






A : ASD large secundum + LPA stenosis moderate + Aortic stenosis moderate

+ Malnutrition marasmic type

P : - O2 1-2 L/i

- F75 diet 60 cc / 3 hours / NGT

- IVFD D5% NaCl 0,225% 4 gtt/i micro

- Inj. Cefotaxime 150 mg/12h/i.v.

- Inj. Ampicillin 150 mg/8h/i.v.

- Paracetamol 3x50 mg

- Lasix 2x5 mg


- Folic acid 1x1 mg

- Zinc 1x10 mg


February 14th 2010

S : Shortness of breath (-), fever(-)







Chest : Symmetrical fusiform, retraction (-). Ribs are clearly visible.



Abdomen : soepel








P : - O2 1-2 L/i




- Inj. Ampicillin 150 mg/8h/i.v


- Lasix 2x5 mg


- Folic acid 1x1 mg

- Zinc 1x10 mg


February 15th 2010 - February 16th 2010

S : Shortness of breath (-), fever(-)







Chest : Symmetrical fusiform, retraction (-). Ribs are clearly visible.



Abdomen : soepel








P : - O2 1-2 L/i






- Lasix 2x5 mg


- Folic acid 1x1 mg

- Zinc 1x10 mg


February 17th 2010 - February 18th 2010

S : Shortness of breath (-), fever( -)







Chest : Symmetrical fusiform, retraction (+) epigastrial. Ribs are clearly visible.



Abdomen : soepel








P : - O2 1-2 L/i






- Lasix 2x5 mg


- Folic acid 1x1 mg

- Zinc 1x10 mg


DISCUSSION

The great majority of infants with an ASD are most often asymptomatic. Frequently,

the condition is detected by a murmur on routine physical examination when they are school-

aged children. Even an extremely large secundum ASD rarely produces clinically evident

heart failure in childhood. In younger children, subtle failure to thrive may be present , in

older children, varying degrees of exercise intolerance may be noted. If symptoms do occur,

they include dyspnoe on exertion, fatique, and recurrent lower respiratory tract infections.

The most common symptoms in adults are decreased stamina and palpitations due to atrial

tachyarrhytmia resulting from right atrial enlargement. Often, the degree of limitation may go

unnoticed by the family until after surgical repair, when the child's growth or activity level

increases markedly.1,5 With a large shunt, there is often a left parasternal budge evident, and

the underlying enlarged right ventricle is palpable. The first heart sound is characteristically

loud at the left lower sternal border (in the presence of a normal or short P-R interval on the

electrocardiogram). The second heart sound at the left upper sternal border is widely split,

with the splitting interval fixed and unaffected by respiration, owing to the large

nonrestrictive atrial defect equalizing the respiratory influence on both right and left

ventricular output, with the wide split resulting from delayed emptying of the enlarged right

ventricle. The intensity of the pulmonary component is almost always normal, reflecting

normal pulmonary pressure and resistance. Murmurs are not loud and may even be absent

occasionally. There is usually an ejection systolic murmur at the left upper sternal border due

to minor relative pulmonary valve stenosis resulting from the increased flow. There is

frequently an early diastolic flow rumble, often of high frequency, at the left lower sternal

border due to increased flow-related tricuspid stenosis.10

This patient is a 6 months old boy with main complaint shortness of breath,

shortness of breath occur usually after drinking milk for 5 minutes. Although many patients

with an ASD are asymptomatic, but this patient present with a symptom disability to

breastfeeding due to shortness of breath. From physical examination systolic murmurs were

confirmed with grade III/6.

On chest radiograph, the heart usually enlarged due to right atrial and right

ventricular dilatation, and there is prominence of the pulmonary artery with increased

pulmonary vascular markings. These signs vary and may not be conspicuous in mild cases.

Cardiac enlargement is often best appreciated on the lateral view because the right ventricle

protrudes anteriorly as its volume increases.1,5 This patient’s chest radiograph shows an

enlargement of his heart or cardiomegaly.

In patients with an ostium secundum defect, the ECG usually shows right-axis

deviation and an rSr′ pattern in the right precordial leads representing enlargement of the RV

outflow tract and incomplete or complete right bundle branch block. An ectopic atrial

pacemaker or first-degree heart block may occur with defects of the sinus venous type. In

ostium primum defect, the RV conduction defect is accompanied by left superior axis

deviation and counterclockwise rotation of the frontal plane QRS loop thought to be due to

displacement and hypoplasia of the left anterior fascicle. Varying degrees of RV and right

atrial (RA) hypertrophy may occur with each type of defect, depending on the height of the

pulmonary artery pressure.6

The echocardiogram shows findings characteristic of right ventricular volume

overload, including an increased right ventricular end-diastolic dimension and flattening and

abnormal motion of the ventricular septum. A normal septum moves posteriorly during

systole and anteriorly during diastole. With right ventricular overload and normal pulmonary

vascular resistance, septal motion is reversed, that is anterior movement in systole or the

motion may be intermediate so that the septum remains straight. The location and size of the

atrial defect are readily appreciated by two-dimensional scanning and may be visualized

directly, with a characteristic brightening of the echo image seen at the edge of the defect (T-

artifact). The shunt is confirmed by pulsed and color flow Doppler, the magnitude and

direction of shunt flow and an estimation of right ventricular systolic pressure can be

determined.1,5

Given the high sensitivity of echocardiography, it is rarely necessary to perform

cardiac catheterization to confirm the presence of an ASD. However, Patients with the

classic features of a hemodynamically significant ASD on physical examination and chest

radiography, in whom echocardiographic identification of an isolated secundum ASD is

made, need not be catheterized before surgical closure, with the exception of an older patient,

in whom pulmonary vascular resistance may be a concern. If pulmonary vascular disease is

suspected, cardiac catheterization confirms the presence of the defect and allows

measurement of the shunt ratio and pulmonary pressure. At catheterization, the oxygen

content of blood from the right atrium will be much higher than that from the superior vena

cava. This feature is not specifically diagnostic because it may occur with partial anomalous

pulmonary venous return to the right atrium, with a ventricular septal defect (VSD) in the

presence of tricuspid insufficiency, with AV septal defects associated with left ventricular to

right atrial shunts, and with aorta to right atrial communications (ruptured sinus of Valsalva

aneurysm). Pressure in the right side of the heart is usually normal, but small to moderate

pressure gradients (<25 mm Hg) may be measured across the right ventricular outflow tract

because of functional stenosis related to excessive blood flow. In children and adolescents,

the pulmonary vascular resistance is almost always normal. The shunt is variable and depends

on the size of the defect, but it may be of considerable volume (as high as 20 L/min/m2).

Cineangiography, performed with the catheter through the defect and in the right upper

pulmonary vein, demonstrates the defect and the location of the right upper pulmonary

venous drainage. Alternatively, pulmonary angiography demonstrates the defect on the

levophase (return of contrast to the left side of the heart after passing through the lungs).1,5

This patient’s echocardiography shows an ASD Secundum L R shunt with

diameter 5-6 mm, and moderate left pulmonary artery (LPA) stenosis. With normal systolic

function of left ventricle and no sign of cardial effusion. Cardiac catheterization not yet

performed on this patient.

Most patients with an ASD remain active and asymptomatic, in asymptomatic

children, the decision to intervene is based on the presence of right-sided heart dilation and a

significant ASD (>5 mm) that shows no sign of spontaneous closure. Shunt fractions are now

rarely measured and are reserved for “borderline” cases. Hemodynamically insignificant

ASDs (Qp/Qs<1.5) do not require closure, with the possible exception of trying to prevent

paradoxical emboli in older patients after a stroke. “Significant” ASDs (Qp/Qs>1.5, or ASDs

associated with right ventricular volume overload) should be closed, especially if device

closure is available and appropriate. For patients with pulmonary hypertension (pulmonary

artery pressure > 2/3 systemic arterial blood pressure or pulmonary arteriolar resistance > 2/3

systemic arteriolar resistance), closure can be recommended if there is a net left-to-right shunt

of at least 1.5:1, evidence of pulmonary artery reactivity when challenged with a pulmonary

vasodilator (e.g., oxygen or nitric oxide), or evidence on lung biopsy (rarely required) that

pulmonary arterial changes are potentially reversible.2

It is reasonable to follow smaller defects in very young patients, which are

generally asymptomatic, medically for a few years because many are likely to close

spontaneously, particularly those smaller than 3 mm. If, however, after some years of

follow-up, echocardiographic evidence of shunting persists and the defect is 5 mm or

larger, then it should be closed. The primary reason for closing an atrial defect is to prevent

pulmonary vascular disease. Closure also reduces the incidence of supraventricular

dysrhytmias (particularly atrial fibrilation), especially when carried out before age 40 years,

although it does not eliminate this problem even when performed in childhood.10

Closure of the secundum defect may be accomplished surgically or by a catheter-

delivered device. Although surgery remains the more common approach, use of the

latter continues to increase as devices, delivery equipment, and technical skill improve.

Surgical techniques have likewise improved such that use of the “ministernotomy” has

become widespread, and complete defect closure by suture or patch is almost always

accomplished. Mortality is virtually zero, complications are few and mostly minor, and

hospital stay has decreased to about 3 days.10

Device closure at catheterization was initially described in 1976, and in those

few original patients after 27 years of follow-up, occlusion remained effective, although

atrial arrhythmias did occur. During the past two decades, a variety of devices and

their modifications have been used, including the clamshell, button, ASDOS, angel wings,

and Amplatzer devices. The longest follow-up has been with the clamshell device, and

although residual leaks were common, these were trivial in most cases, and arm fracture,

albeit frequent, were without sequelae. Complete closure rates with the Amplatzer device

in appropriate patients have been very satisfactory, ranging from 94% to 98%, with few

severe complications.10

Clearly, device closure is more appealing than surgery because of absence of

a thoracotomy scar and shorter hospital stay. in recent reports comparing surgery

and Amplatzer device, defects were often larger in the surgical patients, there were no

deaths with either technique, and closure rates were similar (96% to 100%) and although

complications occurred in both groups, they were more common in the surgical

population. In terms of cost in three series, surgery was clearly more expensive in one,

slightly more in another.10

This patient also has severe malnutrition marasmic type. It may be experienced on

this patient due to his lack of nutrition support from breastfeeding because of an ASD on this

patient and also moderate LPA stenosis made his clinical symptoms of ASD severed.

Treatment for this patient was planning to do closure of the secundum defect by a

catheter-delivered device. But the intervention was postponed because the nutritional status

of this patient. The intervention will be done until nutritional status of this patient normal.

Other management was Lasix 2x5 mg and aldactone 2x6,25 mg

Severe malnutrition was treated with F75 diet 65 cc / 3 hours / NGT, Folic acid 1x5

mg was given for one day and the next 7 day changed with folic acid 1x1mg, zinc 1x10 mg,

multivitamin syr without Fe 1xCth I, Vitamin A 1x100.000 IU. Cefotaxime 150 mg/12h and

Ampicillin 150 mg/8h was given to prevent infection to this patient.

SUMMARY

It has been reported a case of a 6 months old boy with an atrial septal defect. The diagnosis

was established based on anamnesis, clinical sign, symptoms, physical examination, chest

radiograph, and echocardiographic examination. This patient also diagnosed with severe

malnutrition marasmic type. The prognosis of this patient was good, if the defect closure

done in the early life of this patient.

REFERENCES

1. Chen Y, Liberthson RR, Freed MD. Congenital Heart Disease (Atrial Septal

Defect). In: Lilly LS, Eds. Pathophysiology of Heart Disease.3 rded. Philadelpia,

Lippincott Williams & Wilkins; 2003: 355-358

2. Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital Heart Disease.

In: Libby P, Bonow RO, Mann DL, Zipes DP, Eds. Braunwald’s Heart

Disease.8thed. Philadelpia, Saunders Elsevier publisher;2007:1577-1583

3. English RF, Anderson RH, Ettedgui JA. Interatrial Communications. In:

Anderson RH, Baker EJ, Penny D, Redington AN, Rigby ML, Wernovsky G,

Eds. Paediatric Cardiology. 3rded. Philadelpia, Churchill Livingstone Elsevier;

2010: 523-546

4. Park MK. Left-to-Right Shunt Lesions. In: Park MK, Eds. Pediatric Cardiology

for Practitioners. 5thed. Philadelpia, Mosby Elsevier; 2008: 206-212

5. Bernstein D. Acyanotic Congenital Heart Disease. In: Kliegman RM, Behrman

RE, Jenson HB, Stanton BF, Eds. Nelson Textbook of Pediatrics. 18th ed.

Philadelphia, Saunders Elsevier publisher; 2007: Ch426

6. Child JS. Congenital Heart Disease in the Adult. In: Fauci AS, Kasper DL,

Longo DL, Braunwald E, Hauser SL, Jameson JL, et al, Eds. Harrison’s

Principles of Internal Medicine. 17thed. McGraw-Hill; 2008: 1458-1460

7. Kumar V, Abbas AK, Fausto N, Mitchell R. Congenital Heart Disease. In:

Kumar V, Abbas AK, Fausto N, Mitchell R, Eds. Basic Pathology.8 thed.

Saunders Elsevier publisher; 2007: 382-388

8. Carr MR. Atrial Septal Defect General Concepts. 2008. Available at:

http://emedicine.medscape.com/article/889394-overview [Accessed on March

2nd, 2010]

9. Gessner IH. Atrial Septal Defect Ostium Secundum. 2008. Available at:

http://emedicine.medscape.com/article/890991-overview [Accessed on March

2nd, 2010]

10. Keane JF, Geva T, Fyler DC. Atrial Septal Defect. In: Keane JF, Fyler DC, Lock

JE, eds. Nadas’ Pediatric Cardiology. 2nd ed. Philadelphia, Saunders Elsevier

publisher; 2006: 603-616

http://emedicine.medscape.com/article/890991-overview

http://emedicine.medscape.com/article/889394-overview

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