In congenital heart disease with large shunts at the ventricular or arterial level the mean pulmonary artery pressure is increased to systemic levels. In addition, these shunts also increase the pulse pressure (PP) in the pulmonary arteries; the increased pulsatility, acts apart from mean pressure. Increased PP injures the arterial wall from exaggerated distension with each heart beat, producing medial hypertrophy that narrows the lumen, increasing pulmonary vascular resistance--a positive feedback. Thus, pulmonary arterial hypertension (PAH) is progressive and surgical intervention was considered dangerous. However we have observed adults with PAH who are stable and asymptomatic; they had central PAH but the pressure was reduced in the distal pulmonary arteries by valvular or bilateral distal stenosis. We present briefl y two adults: one illustrates the stabilizing effect of damping pulsatility in the pulmonary arteries, and the second illustrates the catastrophic effects of excessive pulsatility from a shunt.

Pulmonary arterial hypertension (PAH) is normally present in the first few days after birth, and positive remodeling occurs rapidly so that after 3 days the pulmonary artery pressure is less than onefifth of the systemic. However, large ventricular septal defects (VSD) and arterial ducts transmit systemic pressure to the right ventricle from birth, creating pulmonary hypertension (PAH) with increased pulsatility. If the VSD is not closed, the PAH will lead eventually to Eisenmenger syndrome, characterized by obstructive lesions in the pulmonary arteries with a marked increase in pulmonary vascular resistance. Most patients with obstructive pulmonary hypertension follow a downhill course with limited life expectancy.

However we have observed a few patients with a large VSD and mean PAH who have done much better; they had dampening of PP in the pulmonary arteries from mild pulmonic stenosis. (Severe stenosis would result in a tetrad physiology with mean pulmonary artery pressures below normal.) We have also observed an adult tetrad of Fallot who received an overly large shunt to the pulmonary circuit resulting in an acute Eisenmenger syndrome. Until recently, patients with marked PAH have not been offered surgical correction because of the high risk based on earlier surgical experience. That has changed with new surgical approaches, assisted by new pharmacologic treatment, with substantial reduction of the PAH. There have also been recent reports that abnormal pulsatility in the systemic circulation causes vascular injury that does not respond to ordinary antihypertensive medications.

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ISSN: 2155-9880

Current Issue: Volume 11, Issue 7

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