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Ostium Primum Atrial Septal Defect

ASD, Ostium Primum Type

The ostium primum atrial septal defect consists of the absence of the interatrial septum in the atrioventricular canal portion, which causes interatrial left to right shunting. The malformation may occur by itself or in association with an inlet ventricular septal defect, as part of an atrioventricular septal defect. When the ventricular septal defect is absent, the atrioventricular valves are part of a single five-leaflet valve that adheres to the interventricular septum. The anterior leaflet of the mitral valve has a cleft as part of this malformation.


Presentation

Symptoms of ostium primum atrial septal defect depend on the magnitude of the shunt and that of the associated mitral regurgitation. Patients may be asymptomatic or may exhibit tachypnea and tachycardia, exacerbated with exercise, but they may also be present at rest. An effort such as feeding leads to diaphoresis, dyspnea and increased work of breathing that ultimately results in failure to thrive, especially if congestive heart failure due to pulmonary congestion is present. The thorax appears hyperinflated, with prominent Harrison grooves.

Symptoms may not appear during infancy, but as the child grows older and consist of exertional dyspnea, palpitations due to atrial fibrillation or flutter, as the atria dilate [1] or to sinus tachycardia that is a compensatory mechanism of heart failure. Sometimes right heart failure signs appear first. Paradoxical embolism may also occur. Cyanosis only appears once Eisenmenger syndrome has developed especially if there is no concomitant pulmonary stenosis.

The clinical examination may show a displaced apical impulse, a palpable dilated pulmonary artery, a widely split second heart sound (S2) with a loud pulmonary component in the presence of pulmonary hypertension and a variety of murmurs: a diastolic rumble due to the large flow through the tricuspid valve, a murmur of tricuspid regurgitation due to the pulmonary hypertension or one of mitral regurgitation due to the anterior leaflet cleft. As left and right atrial pressures equalize, the jugular venous pressure reflects left atrial pressure.

As pulmonary hypertension sets in, the splitting of S2 becomes more narrow and eventually disappears, as does the diastolic tricuspid rumble; the tricuspid regurgitation becomes more audible and a Graham-Steel pulmonary regurgitation diastolic murmur appears.

Atrial Septal Defect
  • Abstract Two-dimensional and Doppler echocardiography were compared with cardiac catheterization and angiography in the preoperative evaluation of ostium primum atrial septal defect.[ncbi.nlm.nih.gov]
  • The ostium primum atrial septal defect is a defect in the atrial septum at the level of the tricuspid and mitral valves.[en.wikipedia.org]
  • Symptoms of ostium primum atrial septal defect depend on the magnitude of the shunt and that of the associated mitral regurgitation.[symptoma.com]
  • The ostium secundum atrial septal defect is the most common atrial septal defect and occurs when the atrial septum is not completely closed during heart development.[remedyland.com]
  • We describe an adult patient with an ostium primum atrial septal defect (ASD) and a patent foramen ovale (PFO) with normal right heart pressures who presented with platypnea and orthodeoxia.[ncbi.nlm.nih.gov]
Down Syndrome
  • Endocardial cushion defects are the most common congenital heart defect that is associated with Down's syndrome.[ipfs.io]
  • Pulmonary vascular obstructive disease may develop in a subset of patients—and those with Down syndrome are at the highest risk. [6] The prognosis is guarded, and morbidity and mortality are high regardless of therapy.[emedicine.com]
  • ASSESSMENT: Atrioventricular canal defect ICD-10-CM coding: Q21.2, Q90.9 Down syndrome, unspecified About Latest Posts John Verhovshek John Verhovshek, MA, CPC, is a contributing editor at AAPC.[aapc.com]
Sepsis
  • Late postoperative deaths occurred in five patients (15%) and were related to myocardial infarction, stroke, hepatic failure, renal failure or sepsis.[onlinejacc.org]
Falling
  • […] in either direction across an atrial septal defect because the compliance of the right and left ventricles is virtually identical. 20, 49, 50 The right ventricle gradually becomes thinner and more compliant than the left ventricle in response to the fall[clinicalgate.com]
Heart Disease
  • Canadian Cardiovascular Society Consensus Conference 2001 update: rec-ommendations for the management of adults with congenital heart disease part III. Can J Cardiol. 2001; 17: 1135–1158. 3. Gatzoulis MA, Freeman MA, Siu SC, Webb GD, Harris L.[pacificejournals.com]
  • The risk of congenital heart disease is higher for children of parents with congenital heart disease, whether in the father or the mother.[mayoclinic.org]
  • […] of Adults With Congenital Heart Disease).[wikidoc.org]
  • From Wikiecho Atrial septal defects (ASD) are a group of congenital heart diseases characterised by defect in the inter atrial septum allowing blood flow from one atrium to the other. They are among the most common congenital heart diseases.[wikiecho.org]
  • Fyfe DA, Kline CH: Fetal echocardiographic diagnosis of congenital heart disease. Pediatr Clin North Am 1990;37:45–67. Chiba Y, Kanzaki T, Kobayashi H, Murakami M, Yutani C: Evaluation of fetal structural heart disease using color flow mapping.[karger.com]
Heart Murmur
  • Patients typically are referred for evaluation of a heart murmur in childhood and generally survive well into adulthood.[emedicine.com]
  • Atrial septal defect signs and symptoms may include: Shortness of breath, especially when exercising Fatigue Swelling of legs, feet or abdomen Heart palpitations or skipped beats Stroke Heart murmur, a whooshing sound that can be heard through a stethoscope[mayoclinic.org]
  • In childhood, heart murmurs typically are normal findings. In fact, the murmur that is associated with an ASD may sound like a normal heart murmur.[clinicaladvisor.com]
  • Your child's doctor may have heard a heart murmur when listening to your child's heart with a stethoscope. The heart murmur is from the abnormal flow of blood through the heart. Your child may need to see a pediatric cardiologist for a diagnosis.[stlouischildrens.org]
Systolic Murmur
  • Persistent apical systolic murmur suggestive of mitral regurgitation was heard in five patients (42%) after the operation. No clinically significant mitral regurgitation was noted in these patients.[ohsu.pure.elsevier.com]
  • In a II and III intercostal space to the left of the sternum, a moderate systolic murmur can be heard as a result of increased flow through the pulmonary valve.[remedyland.com]
  • Murmurs • Pulmonary ejection systolic murmur – Increased pulmonary flow • Tricuspid murmur – is heard over left sternal edge louder on inspiration • Graham-Steell murmur – of pulmonary regurgitation if pulmonary hypertension 13. • Late systolic murmur[slideshare.net]
  • Auscultation typically reveals a grade 2 to 3/6 midsystolic (or ejection systolic) murmur and a widely split, fixed S2 at the upper left sternal border in children.[babiesonline.co.za]
  • Chest auscultation classically reveals an ejection systolic murmur heard at the left upper sternal border, attributed to increased flow across the pulmonary valve rather than blood shunting across the defect itself 7.[radiopaedia.org]

Workup

Chest radiography reveals right heart enlargement and pulmonary congestion with increased pulmonary vascular markings; if the mitral regurgitation is severe, the left heart may also appear enlarged. The pulmonary trunk and proximal right pulmonary artery may be dilated [2].

Echocardiography is the main diagnostic method. The ostium primum atrial septal defect is seen in the apical four chamber view as a lack of substance in the inferior part of the atrial septum. Parasternal and subcostal short axis views are more suitable for the evaluation of the mitral valve cleft and other mitral abnormalities: double orifice or single papillary muscle. Left ventricle outflow tract is long and narrow and may be obstructed by the abnormal mitral valve.

Color Doppler evaluates the direction of the atrial shunt, mitral, tricuspid and pulmonary regurgitations and identifies associated interventricular or left ventricle to right atrium communications.

Pulsed Doppler helps to evaluate systolic pulmonary artery pressure, based on tricuspid regurgitation peak velocity, while continuous Doppler is useful in identifying left ventricular outflow tract obstruction if present.

Transesophageal echocardiography is seldom used in the preoperative evaluation, being reserved for poor acoustic window patients, but is valuable in the intraoperative setting to evaluate mitral stenosis, residual mitral and tricuspid regurgitation or left ventricular outflow tract obstruction. This method also better describes the margins of the defect, usually represented by the septum primum and the atrioventricular valve annulus [3].

The electrocardiogram may reveal sinus rhythm, atrial flutter or fibrillation [4] [5] [6]. The sinus node may be displaced, leading to negative P waves [7]. The right atrium overload, pictured as peaked P wave in leads D II, D III, V1 and V3R and first-degree atrioventricular block may be present. Right ventricle hypertrophy markers or incomplete right bundle branch block may be present. If mitral valve regurgitation is severe, the left ventricle may be hypertrophied and the left atrium may be overloaded and enlarged leading to a P wave duration of more than 0,08 sec and/or inversion in leads V1 or V3.

Cardiac catheterization is seldom necessary in this disease and is reserved for the evaluation of pulmonary vascular obstructive disease and in cases where systemic/pulmonary debt ratio (Qp/Qs0 needs to be invasively calculated. This procedure allows the direct measurement of intracardiac and pulmonary artery pressures and the calculation of pulmonary resistance. In addition, cardiac anatomy and associated malformations such as additional atrial or ventricular septal defects, patent ductus arteriosus, coarctation of the aorta or anomalous pulmonary venous return are best described by this method.

Cardiac magnetic resonance imaging is another reliable method to assess right ventricular size and function and cardiac anatomy, in general [8].

Left Axis Deviation
  • In primum defects left axis deviation is seen in most patients with an axis of -30 degrees and very few patients have right axis deviation.[en.wikipedia.org]
  • In Primum defects left axis deviation is seen in most patients with an axis of -30 degrees and very few patients have right axis deviation.[ipfs.io]
  • These results and the persisting left axis deviation can be explained on the basis of the known anatomy of the atrioventricular conduction system in patients with ostium primum atrial septal defect.[circ.ahajournals.org]
  • Left axis deviation (-20 to -60 deg) is always present. q in I & avL (counterclockwise loop). qRs in v5 & v6 due to LV volume overload. Chest X-ray RA, RV and LV enlargement. LA is inconspicuous despite MR due to presence of ASD.[heartpearls.com]
  • The 12-lead ECG will show an incomplete right bundle branch block and left axis deviation (in contrast to the secundum ASD which has a right axis deviation).[healio.com]
Right Axis Deviation
  • In primum defects left axis deviation is seen in most patients with an axis of -30 degrees and very few patients have right axis deviation.[en.wikipedia.org]
  • In Primum defects left axis deviation is seen in most patients with an axis of -30 degrees and very few patients have right axis deviation.[ipfs.io]
  • Right axis deviation is not observed in ostium primum ASD. Left atrial abnormality. Complete or incomplete RBBB. Prolongation of the PR interval (first degree atrioventricular block). Sinus venosus ASD Right axis deviation.[metealpaslan.com]
  • The 12-lead ECG will show an incomplete right bundle branch block and left axis deviation (in contrast to the secundum ASD which has a right axis deviation).[healio.com]
  • EKG: in ostium secundum defect: right axis deviation & an rSr' pattern. in ostium primum: left axis deviation & superior orientation & counterclockwise rotation of the QRS loop in the frontal plane.[enotes.tripod.com]
Prolonged PR Interval
  • ECG Right axis deviation in 85% of cases Left axis deviation and prolonged PR interval seen in ostium primum Right Atrial enlargement 16.[slideshare.net]
  • Patients with ASD may have a prolonged PR interval.[ijcva.com]
  • Individuals with atrial septal defects may have a prolonged PR interval (a first degree heart block).[marmur.com]
  • Individuals with atrial septal defects may have a prolonged PR interval (a first-degree heart block).[en.wikipedia.org]
Right Bundle Branch Block
  • When a person is suspected of having an ASD based on the findings of an incomplete right bundle branch block with a rSr' or rSR', the frontal plane QRS should be examined.[en.wikipedia.org]
  • Right ventricle hypertrophy markers or incomplete right bundle branch block may be present.[symptoma.com]
  • When you suspect a patient has an ASD based on the findings of an incomplete Right Bundle Branch Block with a rSr' or rSR' the next thing you should do is examine the frontal plane QRS.[ipfs.io]
  • Incomplete or complete right bundle branch block is encountered in most cases.[remedyland.com]
  • The 12-lead ECG will show an incomplete right bundle branch block and left axis deviation (in contrast to the secundum ASD which has a right axis deviation).[healio.com]
Incomplete Right Bundle Branch Block
  • When a person is suspected of having an ASD based on the findings of an incomplete right bundle branch block with a rSr' or rSR', the frontal plane QRS should be examined.[en.wikipedia.org]
  • Right ventricle hypertrophy markers or incomplete right bundle branch block may be present.[symptoma.com]
  • When you suspect a patient has an ASD based on the findings of an incomplete Right Bundle Branch Block with a rSr' or rSR' the next thing you should do is examine the frontal plane QRS.[ipfs.io]
  • The 12-lead ECG will show an incomplete right bundle branch block and left axis deviation (in contrast to the secundum ASD which has a right axis deviation).[healio.com]
  • ECG incomplete right bundle branch block (RBBB morphology with QRS duration between 110-120 ms) increased specificity with crochetage sign in the inferior (II, III, aVF) leads right precordial (V1-3) "defective T waves" describes biphasic morphology,[radiopaedia.org]
First-Degree Atrioventricular Block
  • The right atrium overload, pictured as peaked P wave in leads D II, D III, V1 and V3R and first-degree atrioventricular block may be present. Right ventricle hypertrophy markers or incomplete right bundle branch block may be present.[symptoma.com]
  • Prolongation of the PR interval: first degree atrioventricular block ( less frequent than ostium primum ASD ). Crochetage sign : notched R wave in inferior limb leads ( may disappear after closure of the defect ).[metealpaslan.com]
T Wave Inversion
  • In the ECG above, you can see an example of the rSR' pattern in V1 with a R' greater than S with T wave inversion which is commonly seen in volume overload right ventricular hypertrophy.[en.wikipedia.org]
  • In the ECG above, you can see an example of the rSR' pattern in V1 with a R' greater than S with T wave inversion which is commonly seen in volume overload Right Ventricular Hypertrophy.[ipfs.io]
Prominent A-Wave
  • If MR is present, the findings of ASD may be modified Prominent v wave in JVP LV type apex IE may occur (unlike secundum ASD) ECG Incomplete RBBB pattern in v1 as in secundum ASD.[heartpearls.com]
Biventricular Hypertrophy
  • Katz-Wachtel phenomenon (biventricular hypertrophy pattern) is observed. Dr. Mahmut Gokdemir has donated this ECG to our website. Click here for a more detailed ECG ECG 6. Fiftynine years old woman who was operated for ASD 23 years ago.[metealpaslan.com]

Treatment

  • Transverse sections of the anterior mitral leaflet echo were examined in 11 patients with this malformation who subsequently received surgical treatment.[ncbi.nlm.nih.gov]
  • Please consult your own licensed physician regarding diagnosis and treatment of any medical condition! Please see also our disclaimer . This site complies with the HONcode standard for health information: verify here . Database updated 2019-02-19.[diseasesdatabase.com]
  • The only successful treatment solutions is surgical treatment or artificial closure of the defect. Right time for surgical treatment can only be based on need for surgery and risks of surgery.[remedyland.com]
  • Diagnosis [ edit ] Classification [ edit ] A defect in the ostium primum is occasionally classified as an atrial septal defect, [1] but it is more commonly classified as an atrioventricular septal defect. [2] [3] Treatment [ edit ] Hemodynamically significant[en.wikipedia.org]
  • You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.[rarediseases.info.nih.gov]

Prognosis

  • The long term prognosis is excellent. Pulmonary hypertension with shunt reversal is a contraindication for surgery, however the pulmonary hypertension can frequently be treated with medicines.[en.wikipedia.org]
  • Pulmonary vascular obstructive disease may develop in a subset of patients—and those with Down syndrome are at the highest risk. [6] The prognosis is guarded, and morbidity and mortality are high regardless of therapy.[emedicine.com]
  • Prognosis of atrial septal defect – ostium secundum, ostium primum Patients with small shunt can live a normal life, but with a big shunt they can survive to middle age or more, before it comes to the appearance of pulmonary hypertension or a cardiac[remedyland.com]
  • Complications Congestive heart failure Pulmonary arterial hypertension Prognosis Excellent prognosis if treated Sinus Venosus Atrial Septal Defect with Partial Anomalous Pulmonary Venous Return.[learningradiology.com]

Etiology

  • […] iM StIll stUdInG Published on Mar 28, 2015 ATRIAL SEPTAL DEFECT ASD - CF, ETIOLOGY ,PATHO -PHYSIOLOGY, SIGNS & SYM, MANAGEMENT.....ETC ATRIAL SEPTAL DEFECT ( ASD) 1. ATRIAL SEPTAL DEFECT AKSHAY AGRAWAL 2. CONTENTS 1. Introduction 2. Defination 3.[slideshare.net]
  • However, those who develop a stroke require further workup to identify the etiology. In those where a comprehensive evaluation is performed and an obvious etiology is not identified, they are defined as having a cryptogenic stroke.[en.wikipedia.org]
  • Etiology During normal embryonic development, the right horn of the sinus venosus encompasses the right superior vena cava (SVC) and inferior vena cava (IVC).[emedicine.medscape.com]
  • Ongoing controversies regarding etiology, diagnosis, treatment There are two dominant controversies regarding ostium secundum ASDs.[clinicaladvisor.com]
  • Twenty-five (9%) of the 280 patients included in our series had serious tricuspid valve insufficiency (3 out of 3) The etiology of tricuspid insufficiency, like mitral valve insufficiency, may depend on anatomical changes or mixoid degeneration, but more[revespcardiol.org]

Epidemiology

  • Epidemiology Male : Female 1:2 Most commonly seen in children 8.[slideshare.net]
  • Epidemiology United States data Sinus venosus atrial septal defects represent approximately 1% of congenital cardiac lesions.[emedicine.medscape.com]
  • Epidemiology [ 1 ] ASDs are the third most common congenital heart disease and occur as an isolated anomaly in 5% to 10% of all congenital heart defects.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology 6. Clinical features 7. Investigations 8. Risk Factors 9. Management 3. Introduction  Septal defects are more common eg of non cyanotic heart disease. Most common congenital heart disease in adults.[slideshare.net]
  • Pathophysiology Atrial septal defects allow blood to shunt from the left atrium to the right atrium. Shunting is minimal during infancy, but typically increases with age as the right ventricle becomes more compliant.[vhlab.umn.edu]
  • Pathophysiology In patients with acyanotic atrial septal defect (ASD), the shunt follows the left atrium - right atrium - right ventricle route.[metealpaslan.com]
  • Pathophysiology The more common sinus venosus type defect (often referred to as the "usual type") occurs in the upper atrial septum and is contiguous with the superior vena cava (SVC).[emedicine.medscape.com]

Prevention

  • Follow-up in this series suggests that surgical correction may prevent these developments.[ncbi.nlm.nih.gov]
  • Prevention In most cases, atrial septal defects can't be prevented. If you're planning to become pregnant, schedule a preconception visit with your health care provider. This visit should include: Getting tested for immunity to rubella.[mayoclinic.org]
  • Results were attributed to the prevention of progressive mitral annular dilatation. [9] The Mayo Clinic reviewed the need for reoperation over a 45-year period (1962-2006) and found that when reoperation was required, overall late survival was significantly[emedicine.com]
  • What about preventing endocarditis? Most children with an ASD are not at increased risk for developing endocarditis.[heart.org]

References

Article

  1. Gatzoulis MA, Freeman MA, Siu SC, et al. Atrial arrhythmia after surgical closure of atrial septal defects in adults. N Engl J Med. 1999; 340: 839–846.
  2. Reading M. Chest x-ray quiz: an atrial septal defect. Aust Crit Care. 2000; 13: 96–119.
  3. Tobis J, Shenoda M. Percutaneous treatment of patent foramen ovale and atrial septal defects. J Am Coll Cardiol 2012;60:1722-32.
  4. Heller J, Hagege AA, Besse B, et al. “Crochetage” (notch) on R wave in inferior limb leads: a new independent electrocardiographic sign of atrial septal defect. J Am Coll Cardiol. 1996; 27: 877–882
  5. Zufelt K, Rosenberg HC, Li MD, Joubert GI. The electrocardiogram and the secundum atrial septal defect: a reexamination in the era of echocardiography. Can J Cardiol. 1998; 14: 227–232.
  6. Guray U, Guray Y, Yylmaz MB, et al. Evaluation of P wave duration and P wave dispersion in adult patients with secundum atrial septal defect during normal sinus rhythm. Int J Cardiol. 2003; 91: 75–79.
  7. Jost CH, Connolly HM, Danielson GK, et al. Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients. Circulation. 2005; 112: 1953–1958.
  8. Prasad SK, Soukias N, Hornung T, et al. Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage. Circulation. 2004; 109: 207–214.

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Last updated: 2019-07-11 20:03