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Pulmonary Hypertension

PH resulting from congenital defects

08/02/2007

Question:

My daughter age 27 had a PDA ligation and banding of the pulmonary artery at 5.5 months of age and repair of VSD at 2.5 years of age. She was followed by a cardiologist until adolescence and was told that she had mitral valve prolapse. She saw a cardiologist 2 years ago and he says she does not have MVP but that she has pulmonary hypertension (40), based upon findings of an ECHO. Chest xray shows chronic bilateral pleural and parenchymal scarring. She has incomplete right bundle branch block and right ventricular hypertrophy. All of this according to him is residual from the childhood disease process and all he offers when questioned about this is that we dont know what might happen in the future but probably she will experience heart failure. As she is mentally handicapped because of a chromosone 1 abnormality, along with agenesis of the corpus callosum and colpocephaly, etc., she is unable to verbalize symptoms she may be feeling therefore I am wondering if she is experiencing any. My question is does this pressure usually cause symptoms, is this unusual for her to still have an elevated pressure and also after studying a little about PH I wonder if his assessment is reasonable.

Answer:

When patients with PDA and VSD are not treated early, most develop pulmonary hypertension by 2 yrs of age, though it may be delayed in some patients. In your daughters case, she had surgical correction pretty early and some of the increased pulmonary artery pressures seen on the echo likely are residua from her childhood issues. It is hard say at which pressure people experience symptoms. People who feel short of breath will breathe faster than normal.

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Response by:

Namita   Sood, MD, FCCP Namita Sood, MD, FCCP
Associate Professor of Pulmonary, Critical Care & Sleep
College of Medicine
The Ohio State University