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Pediatrics: Cardiac Disease and Critical Care Medicine

Ostium Primum Atrial Septal Defects

Updated: Aug 08, 2017
  • Author: Shannon M Rivenes, MD; Chief Editor: Syamasundar Rao Patnana, MD  more…
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Sections
Ostium Primum Atrial Septal Defects
  • Sections
    Ostium Primum Atrial Septal Defects
  • Overview
    • Background
    • Pathophysiology
    • Etiology
    • Epidemiology
    • Prognosis
    • Show All
  • Presentation
    • History
    • Physical Examination
    • Show All
  • DDx
  • Workup
    • Imaging Studies
    • Other Tests
    • Procedures
    • Show All
  • Treatment
    • Approach Considerations
    • Medical Care
    • Surgical Care
    • Long-Term Monitoring
    • Show All
  • Medication
    • Medication Summary
    • Diuretic
    • Angiotensin-converting enzyme inhibitors
    • Show All
  • Media Gallery
  • References

Overview

Background

An ostium primum atrial septal defect (ASD), as seen in the image below, is located in the most anterior and inferior aspect of the atrial septum. It is the simplest form of atrioventricular (AV) canal or AV septal defect. These defects are often associated with trisomy 21.

Gross pathology specimen viewed from the opened le

Gross pathology specimen viewed from the opened left atrium and left ventricle, demonstrating a partial atrioventricular (AV) canal defect. An ostium primum atrial septal defect (ASD) marked by an asterisk (*) is visualized in the inferior aspect of the interatrial septum. An ostium secundum ASD marked by two asterisks (**) is also noted. The mitral valve is cleft and the leaflets are thickened and rolled, suggestive of chronic mitral regurgitation. LA = left atrium, LV = left ventricle, and MV = mitral valve.

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During fetal development, the rudimentary atrium is divided by the septum primum, except for an anterior and inferior space that is the ostium primum. The ostium primum is sealed by fusion of the superior and inferior endocardial cushions around 5 weeks’ gestation. Failure to do so results in an ostium primum ASD. Observations by Anderson and colleagues suggest that failure of growth of the vestibular spine to complete atrial septation may result in the ostium primum atrial defect.
[ 1 ]

The endocardial cushions also contribute to the complete formation of two separate AV valves and the inlet interventricular septum. For this reason, ostium primum ASDs are commonly associated with malformations of these structures. 

Ostium primum ASDs are most commonly seen with a cleft in the anterior leaflet of the mitral valve, but they may occur in isolation. This is sometimes termed a partial AV canal defect or a partial AV septal defect. In this case, a five-leaflet AV valve is arranged so that separate right and left components (a tricuspid valve and a mitral valve) are present. The leaflets connect to each other and then adhere to the crest of the interventricular septum. This results in shunting at the atrial level with no ventricular level shunting. Generally, a commissure is observed between the left superior and inferior bridging leaflets because of abnormal fusion of the left tubercle of the superior and inferior cushions, which results in a cleft in the anterior leaflet of the mitral valve.

For patient education resources, see  Heart Health Center , as well as  Palpitations .

Next:

Pathophysiology

Shunting is predominantly left-to-right in the absence of pulmonary vascular disease or significant right ventricular outflow tract obstruction. This results in volume overload of the right atrium and ventricle and pulmonary overcirculation. If the mitral valve cleft causes significant mitral regurgitation, the left side of the heart also becomes volume overloaded. A left ventricle-to-right atrium shunt can be present, which further overloads both the right and left heart.

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Etiology

The most common association of an ostium primum atrial septal defect (ASD) is genetic, associated with trisomy 21. Well-described associations have also been reported with  Holt-Oram syndrome ,  Noonan syndrome , and  Ellis-van Creveld syndrome , among others. In children with normal chromosomes, however, the cause remains unknown. Research into the molecular genetic basis for atrioventricular (AV) canal and AV septal defects is ongoing.

A study by Rana et al implicated the TBX1 gene in the development of ostium primum ASDs, among other congenital heart defects. According to these investigators, TBX1 -null embryos are impaired in the ability of second heart field cells (multipotent cardiovascular progenitor cells) to be added to the venous pole of the heart, causing ostium primum defects, as well as abnormal development of the dorsal mesenchymal protrusion.
[ 2 ]

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Next:

Epidemiology

United States statistics

Ostium primum atrial septal defects (ASDs) are most commonly associated with Down syndrome (trisomy 21). The incidence of trisomy 21 is 1 per 800 live births, with an increased prevalence observed in children born to older mothers. Note the following:

  • The overall risk of congenital heart disease in patients with Down syndrome is 40-50%. Approximately 65% of those affected have some form of atrioventricular (AV) septal defect.

  • The inherited risk for children of parents who have an AV septal defect is reported as 9-14%.

Sex- and age-related demographics

The male-to-female ratio is 1:1.

Patients with ostium primum ASD typically present at a young age. Patients very rarely reach adulthood without surgical correction.
[ 3 , 4 , 5 ]

Patients with smaller defects and little or no mitral regurgitation may present at any age with a murmur and/or an abnormal electrocardiogram. Those with more severe mitral regurgitation typically present with congestive heart failure in the first 1-2 years of life.

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Next:

Prognosis

Pediatric patients with small left-to-right shunts and no significant mitral regurgitation who have not undergone surgery are at relatively low risk for complications. In these patients, adult survival is expected, but complications can develop as age advances. Untreated patients with large shunts and/or significant mitral regurgitation are at significant risk of morbidity and mortality. Death, arrhythmia, heart block, refractory heart failure, and advanced pulmonary vascular disease are the most common complications and tend to increase with advancing age. 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.

Surgical repair generally improves life expectancy and alters the natural course of the disease. Investigators at the Hospital for Sick Children in Toront evaluated the long-term outcome of 180 children with ostium primum atrial septal defects (ASDs) from 1982 to 1996 and found that age and preoperative moderate-to-severe left atrioventricular (AV) valve regurgitation were predictors of reoperation, and age at repair younger than 1 year was a predictor of death.
[ 7 ]
 Three patients (1.6%) suffered early mortality, two of whom were infants. Another 17 patients (9%) underwent reoperation (five infants); five patients underwent reoperation for subaortic obstruction, and 12 for left AV valve regurgitation (one required valve replacement, 11 were repaired). Actuarial survival was 98% at 10 years with no late deaths. At 10 years, the actuarial survival was 98% and no late deaths occurred.
[ 7 ]

A study from Oregon Health Sciences University that assessed 38 consecutive patients aged 3-58 months who underwent surgical correction for partial AV septal defect between 1981 and 1997 concluded that an aggressive approach to early operative intervention is safe and effective, as well as provides good long-term results.
[ 8 ]
 Nearly all the patients (92%) underwent closure of the mitral cleft, with 28% of these patients also requiring mitral annuloplasty. Early 30-day mortality was 7.9%. At follow-up up to 14 years, late mitral regurgitation was present in 0.9% of the patients, and there was only one late reoperation. At last follow-up, 13% of the patients were symptomatic.
[ 8 ]

Data from Italian investigators supported the conclusions of Oregon Health Sciences University researchers. Michielon et al documented 93.5% (±2%) freedom from reoperation at 12.3 years for partial AV canal defects.
[ 9 ]
 Reintervention was highest in patients with preoperative AV valve regurgitation and a double orifice left AV valve but was statistically lower for patients who underwent early repair using a bifoliate approach. 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 reduced.
[ 10 ]
Ninety-six patients underwent reoperation (median interval, 10 years), with a median age at first reoperation of 26 years (range, 10 months to 71 years). Indications included left AV valve (LAVV) regurgitation in 67% of patients, subaortic stenosis in 25% of patients, right AV valve regurgitation in 22% of patients, residual ASD in 11% of patients, and other indications in 6%. Of the five early deaths, three occurred prior to 1983. No significant difference was noted in 20-year survival after LAVV repair or replacement (69% vs 55%, P = 0.20). At last follow-up (median, 5.2 years; max, 34 years), 81 of 89 late survivors had New York Heart Association (NYHA) functional class I or II.
[ 10 ]

The Mayo Clinic also previously reviewed the adult experience of 31 patients aged 40-71 years at the time of repair at their institution from 1958 to 1990
[ 11 ]
; 23 had repair of the mitral cleft, two required mitral valve replacements, and six warranted mitral reoperation. Early mortality was 6%; 19 patients were followed for a mean of 14 years, with 14 reporting a sustained postoperative improvement.
[ 11 ]

The Pediatric Heart Network evaluated the need for reoperation for partial versus other forms of ASDs in 215 patients from seven North American centers and found that the subtype of AV septal defect was significantly associated with preoperative patient characteristics and clinical status as well as influenced the age at repair.
[ 12 ]
Patients were subtyped as partial (n = 60), transitional (n = 27), complete (n = 120), and canal-type ventricular septal defect (VSD) (n = 8). The highest preoperative prevalence of moderate or severe LAVV regurgitation occurred in those with transitional ASD (P = 0.01).
[ 12 ]

Significant postoperative LAVV regurgitation was the most common sequela (similar prevalence across all centers at 6 months).
[ 12 ]
At 6-month follow-up, older age at repair was an independent predictor of moderate or severe LAVV regurgitation (P = 0.02) but not annuloplasty, subtype, or center (P >0.4). After accounting for age at repair, there was no association seen between AV septal defect subtype and postoperative LAVV regurgitation severity or growth failure at 6 months.
[ 12 ]
 Annuloplasty failed to decrease the postoperative prevalence of moderate or severe LAVV regurgitation at 6 months

Morbidity/mortality

The presence and degree of associated mitral regurgitation and/or left ventricle-to-right atrium shunting generally determine the symptoms.

Patients with either no cleft or a cleft with a mild degree of mitral regurgitation are often asymptomatic. Patients typically are referred for evaluation of a heart murmur in childhood and generally survive well into adulthood. However, adults who have not had the condition repaired often become symptomatic from  congestive heart failure (CHF)  by age 45 years.
[ 13 ]
Rarely, patients are reported to present in the seventh decade of life.
[ 14 ]
Dyspnea on exertion and fatigue are the usual adult complaints, as are palpitations secondary to atrial fibrillation or flutter.

Those with more severe mitral regurgitation or left ventricle-to-right atrium shunting often present in the first 2 years of life. Mortality has been reported to be as high as 30% in this subpopulation in the first year of life.

Although relatively rare, pulmonary vascular obstructive disease may occur in patients with long-standing substantial shunts and significant mitral regurgitation.

As noted earlier, children with trisomy 21 are at higher risk than the general population of developing pulmonary vascular obstructive disease at a younger age. Potential reasons for this include chronic upper airway disease, tonsillar and adenoid hypertrophy, and inadequate alveolarization of the terminal bronchioles, leading to a decreased surface area of the vascular bed.

Complications

Infective endocarditis remains both a preoperative and a postoperative complication. In a study from Oregon Health Sciences University, the 30-year postoperative incidence of infective endocarditis was 2.8% among patients with ostium primum ASDs.
[ 15 ]

 

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Clinical Presentation
 

 
References

  1. Anderson RH, Mohun TJ, Brown NA. Clarifying the morphology of the ostium primum defect. J Anat. 2015 Mar. 226 (3):244-57. [Medline] .

  2. Rana MS, Theveniau-Ruissy M, De Bono C, et al. Tbx1 coordinates addition of posterior second heart field progenitor cells to the arterial and venous poles of the heart. Circ Res. 2014 Oct 10. 115 (9):790-9. [Medline] .

  3. Di Gioia G, Mega S, Miglionico M, Di Sciascio G. Large ostium primum interatrial septum defect in asymptomatic elderly patient. J Cardiovasc Echogr. 2016 Jan-Mar. 26 (1):16-8. [Medline] .

  4. Mandal K, Srivastava AR, Nifong LW, Chitwood WR Jr. Robot-assisted partial atrioventricular canal defect repair and Cryo-Maze procedure. Ann Thorac Surg. 2016 Feb. 101 (2):756-8. [Medline] .

  5. Meka SG, Shelden D, Mertens A, Christensen P, Patel M. A hol(e)y predicament. Respirol Case Rep. 2017 Jul. 5 (4):e00237. [Medline] .

  6. Sinha R, Thangaswamy CR, Muthiah T, Chandra P, Subramaniam R. Prolonged postoperative desaturation in a child with Down syndrome and atrial septal defect. Indian J Anaesth. 2011 Nov. 55 (6):608-10. [Medline] . [Full Text] .

  7. Najm HK, Williams WG, Chuaratanaphong S, Watzka SB, Coles JG, Freedom RM. Primum atrial septal defect in children: early results, risk factors, and freedom from reoperation. Ann Thorac Surg. 1998 Sep. 66 (3):829-35. [Medline] .

  8. Agny M, Cobanoglu A. Repair of partial atrioventricular septal defect in children less than five years of age: late results. Ann Thorac Surg. 1999 May. 67 (5):1412-4. [Medline] .

  9. Michielon G, Stellin G, Rizzoli G, et al. Left atrioventricular valve incompetence after repair of common atrioventricular canal defects. Ann Thorac Surg. 1995 Dec. 60 (6 Suppl):S604-9. [Medline] .

  10. Stulak JM, Burkhart HM, Dearani JA, et al. Reoperations after repair of partial atrioventricular septal defect: a 45-year single-center experience. Ann Thorac Surg. 2010 May. 89 (5):1352-9. [Medline] .

  11. Bergin ML, Warnes CA, Tajik AJ, Danielson GK. Partial atrioventricular canal defect: long-term follow-up after initial repair in patients > or = 40 years old. J Am Coll Cardiol. 1995 Apr. 25 (5):1189-94. [Medline] .

  12. Kaza AK, Colan SD, Jaggers J, et al, for the Pediatric Heart Network Investigators. Surgical interventions for atrioventricular septal defect subtypes: the Pediatric Heart Network experience. Ann Thorac Surg. 2011 Oct. 92 (4):1468-75; discussion 1475. [Medline] . [Full Text] .

  13. Ruivo C, Guardado J, Montenegro Sa F, et al. Gerbode defect and multivalvular dysfunction: complex complications in adult congenital heart disease. Echocardiography. 2017 Jul. 34 (7):1099-101. [Medline] .

  14. Meka SG, Shelden D, Mertens A, Christensen P, Patel M. A hol(e)y predicament. Respirol Case Rep. 2017 Jul. 5 (4):e00237. [Medline] .

  15. Morris CD, Reller MD, Menashe VD. Thirty-year incidence of infective endocarditis after surgery for congenital heart defect. JAMA. 1998 Feb 25. 279 (8):599-603. [Medline] .

  16. Martin SS, Shapiro EP, Mukherjee M. Atrial septal defects – clinical manifestations, echo assessment, and intervention. Clin Med Insights Cardiol. 2014. 8 (suppl 1):93-8. [Medline] .

  17. Gil-Jaurena JM, Zabala JI, Conejo L, et al. Minimally invasive pediatric cardiac surgery. Atrial septal defect closure through axillary and submammary approaches. Rev Esp Cardiol. 2011 Mar. 64 (3):208-12. [Medline] .

  18. Murashita T, Kubota T, Oba J, Aoki T, Matano J, Yasuda K. Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect. Ann Thorac Surg. 2004 Jun. 77 (6):2157-62. [Medline] .

  19. [Guideline] Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. J Am Dent Assoc. 2007 Jun. 138 (6):739-45, 747-60. [Medline] . [Full Text] .

  20. [Guideline] Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Thorac Cardiovasc Surg. 2014 Jul. 148 (1):e1-e132. [Medline] . [Full Text] .

  21. [Guideline] Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2017 Jul 11. 70 (2):252-89. [Medline] . [Full Text] .

  22. Aeba R, Kudo M, Okamoto K, Yozu R. Bridging annuloplasty for left atrioventricular valve of partial atrioventricular septal defect. Ann Thorac Surg. 2012 May. 93 (5):e137-9. [Medline] .

  23. Cheitlin MD, Douglas PS, Parmley WW. 26th Bethesda conference: recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities. Task Force 2: acquired valvular heart disease. J Am Coll Cardiol. 1994 Oct. 24 (4):874-80. [Medline] .

  24. Del Nido PJ, Bichell DP. Minimal-access surgery for congenital heart defects. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 1998. 1:75-80. [Medline] .

  25. Giamberti A, Mazzera E, Di Chiara L, Ferretti E, Pasquini L, Di Donato RM. Right submammary minithoracotomy for repair of congenital heart defects. Eur J Cardiothorac Surg. 2000 Dec. 18 (6):678-82. [Medline] .

  26. Graham TP Jr, Bricker JT, James FW, Strong WB. 26th Bethesda conference: recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities. Task Force 1: congenital heart disease. J Am Coll Cardiol. 1994 Oct. 24 (4):867-73. [Medline] .

  27. Kaur A, Srivastava S, Lytrivi ID, Nguyen K, Lai WW, Parness IA. Echocardiographic evaluation and surgical implications of common atrioventricular canal defects with absent or diminutive ostium primum defect. Am J Cardiol. 2008 Jun 1. 101 (11):1648-51. [Medline] .

  28. Lange A, Mankad P, Walayat M, Palka P, Burns JE, Godman MJ. Transthoracic three-dimensional echocardiography in the preoperative assessment of atrioventricular septal defect morphology. Am J Cardiol. 2000 Mar 1. 85 (5):630-5. [Medline] .

  29. Marino B, Digilio MC, Toscano A, Giannotti A, Dallapiccola B. Congenital heart diseases in children with Noonan syndrome: an expanded cardiac spectrum with high prevalence of atrioventricular canal. J Pediatr. 1999 Dec. 135 (6):703-6. [Medline] .

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  31. Pretre R, Dave H, Kadner A, Bettex D, Turina MI. Direct closure of the septum primum in atrioventricular canal defects. J Thorac Cardiovasc Surg. 2004 Jun. 127 (6):1678-81. [Medline] .

  32. Sadler TW. Langman’s Medical Embryology. 5th ed. Baltimore, MD: Williams & Wilkins; 1985. 176-84.

  33. Snider AR, Serwer GA, Ritter SB. Echocardiography in Pediatric Heart Disease. 2nd ed. St Louis, MO: Harcourt Health Sciences Group; 1997. 277-89.

  34. Zanchetta M, Rigatelli G, Pedon L, Zennaro M, Maiolino P, Onorato E. Role of intracardiac echocardiography in atrial septal abnormalities. J Interv Cardiol. 2003 Feb. 16 (1):63-77. [Medline] .

  35. Devlin PJ, Backer CL, Eltayeb O, Monge MC, Hauck AL, Costello JM. Repair of Partial Atrioventricular Septal Defect: Age and Outcomes. Ann Thorac Surg. 2016 Jul. 102 (1):170-7. [Medline] .

Media Gallery
  • Electrocardiogram from a patient with a partial atrioventricular septal defect. The PR interval is mildly prolonged. Left axis deviation with Q waves in leads I and aVL are present, consistent with a counterclockwise loop in the frontal plane. Right atrial enlargement and an rsR’ pattern in the right chest leads are also noted.
  • Two-dimensional, apical, four-chamber echocardiogram of a partial atrioventricular (AV) canal defect. The asterisk (*) delineates an area of dropout in the inferior atrial septum at the site of the primum atrial septal defect. The AV valves are separate but aligned at the same horizontal level, consistent with a two-orifice common AV valve. In systole, the medial leaflets of the right- and left-sided AV valves demonstrate attachments to the crest of the interventricular septum, allowing no ventricular level shunting. LA = left atrium, LV = left ventricle, RA = right atrium, and RV = right ventricle.
  • Gross pathology specimen viewed from the opened left atrium and left ventricle, demonstrating a partial atrioventricular (AV) canal defect. An ostium primum atrial septal defect (ASD) marked by an asterisk (*) is visualized in the inferior aspect of the interatrial septum. An ostium secundum ASD marked by two asterisks (**) is also noted. The mitral valve is cleft and the leaflets are thickened and rolled, suggestive of chronic mitral regurgitation. LA = left atrium, LV = left ventricle, and MV = mitral valve.

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    Contributor Information and Disclosures

    Author

    Shannon M Rivenes, MD Assistant Professor, Department of Pediatrics, Division of Pediatric Cardiology, Texas Children’s Hospital and Baylor College of Medicine

    Shannon M Rivenes, MD is a member of the following medical societies: Alpha Omega Alpha , American Academy of Pediatrics , American College of Cardiology , American Heart Association , American Society of Echocardiography

    Disclosure: Nothing to disclose.

    Specialty Editor Board

    Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

    Disclosure: Nothing to disclose.

    Alvin J Chin, MD Emeritus Professor of Pediatrics, University of Pennsylvania School of Medicine

    Disclosure: Nothing to disclose.

    Chief Editor

    Syamasundar Rao Patnana, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children’s Memorial Hermann Hospital

    Syamasundar Rao Patnana, MD is a member of the following medical societies: American Academy of Pediatrics , American Pediatric Society , American College of Cardiology , American Heart Association , Society for Cardiovascular Angiography and Interventions , Society for Pediatric Research

    Disclosure: Nothing to disclose.

    Additional Contributors

    Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children’s Hospital

    Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics , American College of Cardiology , American Heart Association , Arkansas Medical Society , International Society for Heart and Lung Transplantation , Society for Cardiovascular Angiography and Interventions

    Disclosure: Nothing to disclose.

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    • Sections
      Ostium Primum Atrial Septal Defects
    • Overview
      • Background
      • Pathophysiology
      • Etiology
      • Epidemiology
      • Prognosis
      • Show All
    • Presentation
      • History
      • Physical Examination
      • Show All
    • DDx
    • Workup
      • Imaging Studies
      • Other Tests
      • Procedures
      • Show All
    • Treatment
      • Approach Considerations
      • Medical Care
      • Surgical Care
      • Long-Term Monitoring
      • Show All
    • Medication
      • Medication Summary
      • Diuretic
      • Angiotensin-converting enzyme inhibitors
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    Ostium primum atrial septal defect

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    Ostium primum atrial septal defect
    Schematic drawing of various types of atrial septal defect.png
    Schemating drawing showing the location of different types of ASD, the view is into an opened right atrium. HV: right ventricle; VCS: superior caval vein; VCI: inferior caval vein; 1: upper sinus venosus defect; 2: lower sinus venosus defect; 3: secundum defect; 4: defect involving coronary sinus; 5; primum defect.
    Specialty Medical genetics   Edit this on Wikidata

    The ostium primum atrial septal defect (also known as an endocardial cushion defect) is a defect in the atrial septum at the level of the tricuspid and mitral valves. This is sometimes known as an endocardial cushion defect because it often involves the endocardial cushion , which is the portion of the heart where the atrial septum meets the ventricular septum and the mitral valve meets the tricuspid valve.

    Endocardial cushion defects are associated with abnormalities of the atrioventricular valves (the mitral valve and the tricuspid valve ). These include the cleft mitral valve, and the single atrioventricular valve (a single large, deformed valve that flows into both the right ventricle and the left ventricle).

    Endocardial cushion defects are the most common congenital heart defect that is associated with Down’s syndrome .

    Contents

    • 1 Signs and symptoms
    • 2 Diagnosis
      • 2.1 Classification
    • 3 Treatment
    • 4 References
    • 5 External links

    Signs and symptoms[ edit ]

    On ECG a left axis deviation is generally found in ostium primum ASD, but an RSR pattern (M pattern) in V1 is characteristic. Fixed splitting of the second heart sound (S2) occurs because of equal filling of the left and right atria during all phases of the respiratory cycle.

    ECG of a patient with Ostium primum ASD

    Patients with atrial Septal Defects may have atrial fibrillation , atrial tachycardia , or atrial flutter , but these abnormal heart rhythms are not usually seen until the affected individual grows older. Features also seen on the ECG include right atrial enlargement and varying degrees of atrioventricular 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. The frontal plane QRS is the most helpful clue to distinguish between an ostium secundum ASD and an ostium primum ASD. 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. In contrast ostium secundum defects have an axis between 0 degrees and 180 degrees with most cases to the right of 100 degrees.

    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.

    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 ASDs (flow ratio 1.5:1) are large enough to be closed surgically. The long term prognosis is excellent. Shunt reversal is a contraindication for surgery.

    References[ edit ]

    1. ^ “Atrial Septal Defect Types – Mayo Clinic” . Retrieved 2007-10-14.

    2. ^ Fix, James D.; Dudek, Ronald W. (1998). Embryology. Baltimore: Williams & Wilkins. p. 52. ISBN   0-683-30272-8 .
    3. ^ Q21.2
    • Pryor R, Woodwork MB, Blount SG: Electrocardiographic Changes in Atrial Septal Defects:Ostium Secundum versus Ostium Primum defect. Am Heart J 58:689, 1959.

    External links[ edit ]

    Classification
    D
    • ICD – 10 : Q21.2
    • ICD – 9-CM : 745.6
    • DiseasesDB : 1090
    External resources
    • eMedicine : ped/1685

     This article incorporates text available under the CC BY-SA 3.0 license.

    • v
    • t
    • e
    Congenital heart defects ( Q20–Q24 , 745–746 )
    Cardiac shunt /
    heart septal defect
    Aortopulmonary septal defect
    • R→L: Double outlet right ventricle
      • Taussig–Bing syndrome
    • Transposition of the great vessels
      • dextro
      • levo
    • Persistent truncus arteriosus
    • Aortopulmonary window
    Atrial septal defect
    • L→R: Sinus venosus atrial septal defect
    • Lutembacher’s syndrome
    Ventricular septal defect
    • L→R and R→L: Eisenmenger’s syndrome
    • R→L, with other conditions: Tetralogy of Fallot
    Atrioventricular septal defect
    • L→R: Ostium primum
    Valvular heart disease /
    heart chambers
    Right
    • pulmonary valves
      • stenosis
      • insufficiency
      • absence
    • tricuspid valves
      • stenosis
      • atresia
      • Ebstein’s anomaly
    • Hypoplastic right heart syndrome
      • Uhl anomaly
    Left
    • aortic valves
      • stenosis
      • insufficiency
      • bicuspid
    • mitral valves
      • stenosis
      • regurgitation
    • Hypoplastic left heart syndrome
    Other
    • Dextrocardia
    • Levocardia
    • Cor triatriatum
    • Crisscross heart
    • Brugada syndrome
    • Coronary artery anomaly
    • Anomalous aortic origin of a coronary artery
    • Ventricular inversion

    Retrieved from ” https://en.wikipedia.org/w/index.php?title=Ostium_primum_atrial_septal_defect&oldid=849901350 ”
    Categories :

    • Congenital heart defects
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    • Infobox medical condition (new)
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        • Pediatrics Chapter
        • Ostium Primum

        Ostium Primum

        Aka: Ostium Primum

        Cardiovascular Medicine

        Pediatrics Chapter

        • Coronary Artery Disease
        • Kawasaki Disease
        • CHD
        • Congenital Heart Disease
        • Congenital Heart Disease Causes
        • Atrial Septal Defect
        • Ostium Primum
        • Ostium Secundum
        • Endocardial Cushion Defect
        • Patent Ductus Arteriosus
        • Patent Foramen Ovale
        • Ventricular Septal Defect
        • Anomalous Pulmonary Venous Return
        • Ebstein’s Anomaly
        • Eisenmenger’s Complex
        • Hypoplastic Left Heart Syndrome
        • Tetralogy of Fallot
        • Transposition of the Great Vessels
        • Truncus Arteriosus
        • Dextrocardia
        • Vascular Ring
        • Congestive Heart Failure
        • Pediatric Congestive Heart Failure
        • Hypertension
        • Hypertension in Children
        • Symptoms
        • Pediatric Syncope
        • Circulatory Disorders
        • Aortic Coarctation

        From Related Chapters

        • Examination
        • Pediatric Murmur
        • Physiologic PPS
        • Pulmonary Flow Murmur
        • Still’s Murmur
        • Supraclavicular Murmur
        • Venous Hum
        • Pediatric Vital Signs

        • See Also
        • Page Contents
        • Endocardial Cushion Defect
        • Atrial Septal Defect
        • Congenital Heart Disease
        • Congenital Heart Disease Causes
        • Page Contents…
        • Epidemiology
        • Pathophysiology
        • Symptoms
        • Signs
        • Radiology: Chest XRay
        • Diagnostics: Electrocardiogram (EKG)
        • References
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        II. Epidemiology

        1. Incidence : 2% of Congenital Heart Defect s

        III. Pathophysiology

        1. One of three types of Atrial Septal Defect
        2. Congenital opening in septum near AV valves
        3. Defect occurs during Embryo nic development
        4. Associated with cleft mitral valve leaflet

        IV. Symptoms

        1. Dyspnea

        V. Signs

        1. Rounded chest
        2. Precordial bulge (rare)
        3. No Cyanosis or finger Clubbing
        4. Accentuated precordial Apical Thrust
        5. Accentuated P2 with fixed splitting on respirations
        6. Systolic thrill near sternal border
        7. Systolic Murmur

          1. Harsh Systolic Murmur at left sternal border
          2. Apical Systolic Murmur transmitted to axilla
            1. Mitral Regurgitation
        8. Mid- Diastolic Murmur at apex

        VI. Radiology: Chest XRay

        1. Cardiomegaly

        VII. Diagnostics: Electrocardiogram (EKG)

        1. Incomplete Right Bundle Branch Block

        VIII. References

        1. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]
        2. Cyran (1998) PREP review lecture, October, Phoenix
        3. Merenstein (1994) Pediatrics, Lange

        Images: Related links to external sites (from Bing)

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        Related Studies (from Trip Database) Open in New Window

        Ontology:
        Structure of ostium primum
        (C0225839)

        Concepts Body Part, Organ, or Organ Component
        (T023)

        SnomedCT

        86023005
        English ostium primum, Ostium primum, Structure of ostium primum (body structure), Structure of ostium primum
        Spanish estructura del ostium primum (estructura corporal), estructura del ostium primum, ostium primum
        Derived from the NIH UMLS ( Unified Medical Language System )

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