Atrial septal defect pulmonary hypertension

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Ostium Secundum Atrial Septal Defect
Ostium Primum Atrial Septal Defect
Sinus Venosus Atrial Septal Defect
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Priyamvada Singh, MBBS [2]; Cafer Zorkun, M.D., Ph.D. [3]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [4]

Overview

Atrial septal defect patients who develop severe fixed pulmonary hypertension have poor prognosis irrespective of medical or surgical therapies compared to patients with normal pulmonary artery pressure.

Pulmonary Hypertension

Patients with pulmonary hypertensive conditions face additional challenges in the management of atrial septal defect.

  • In patients with severe fixed pulmonary hypertension (pulmonary vascular resistance is ≥ 15 Wood units) the prognosis is not very good despite medical or surgical therapy.
  • On the other hand, an inter-atrial communication is considered advantageous in this setting conditions as the interatrial communication may help in decompressing the right side of heart.
  • If severe pulmonary hypertension is present, surgical, medical, and natural disease progression all have similarly high mortality rates.
  • Patients with less severe forms of pulmonary hypertension have more favorable results with medical and surgical therapy.
  • On the other hand, individuals with a PVR of ≥ 15 wood units have increased mortality associated with closure of the ASD.
  • Surgical mortality due to closure of an ASD is lowest when the procedure is performed prior to the development of significant pulmonary hypertension.
  • The lowest mortality rates are achieved in individuals with a pulmonary artery systolic pressure of ≤ 40 mm Hg.

Supportive Trial Data

In a study done by Steele et al of 702 patients over 25 years, with atrial septal defects (secundum or sinus venosus type) forty patients (6%) had pulmonary vascular obstructive disease. Of these 40 patients, 26 underwent surgical closure and 14 received medical treatment. The patients were followed for a median follow up of 12 years. During follow-up, less than half patients died. Of the 22 surgically treated patients who had a total pulmonary vascular resistance of less than 15 U/m2, 19 were alive with substantial improvements in the symptoms. The four surgically treated patients who had a pulmonary resistance greater than or equal to 15 U/m2 died during the follow-up period. The outcome of medically treated patient was worse compared to the surgically corrected patients as 4 out of 5 patients with pulmonary resistance less than 15 U/m2 died. The one patient that was living had significant progression of symptoms. The other nine medically treated patients with pulmonary resistance greater than or equal to 15 U/m2, 67% (6) died and the remaining survivors had progression of symptoms.[1]

References

  1. Steele PM, Fuster V, Cohen M, Ritter DG, McGoon DC (1987). "Isolated atrial septal defect with pulmonary vascular obstructive disease--long-term follow-up and prediction of outcome after surgical correction". Circulation. 76 (5): 1037–42. PMID 3664992.

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