15 February 2020 by Susan Pumacayo

Unilateral pulmonary hypertension, in a rare congenital heart disease diagnosis and later treatment

The anomalous origin of one pulmonary artery (AOPA) from the ascending aorta (AAo), the incidence is 0.1% of all CHD. Requiring immediate surgical correction to avoid progressive pulmonary hypertension and congestive heart failure.

A 43-year-old male patient, who at 9 years of age was diagnosed with PDA and pulmonary hypertension, the PDA was surgically closed. At 34 years of age he presented hemoptysis. At 43 years of age, he has another episode of hemoptysis and moderate chest pain. A tomography was performed, and an anomalous origin of the RPA from AAo (AORPA) and bronchiectasis in the right lung was found. The Echocardiography showed normal size of left and right ventricles with normal systolic function of both ventricles and AORPA. Cardiac catheterization (Figure 1 - see full pdf) reported systolic/diastolic pressure of LPA was 50/25 mmHg (mean 33), systolic/diastolic pressure of RPA was 120/60mmHg (mean 80), same as systemic pressure, a oxygen vasoreactivity test (OVT) was performed without significant variation. It was decided surgically implantation of the RPA to the MPA with a 16 mm diameter woven dacron tube. One month after surgery a heart catheterization was performed that concludes: pulmonary pressure(PP) of 119/49 mmHg (mean77mmHg), PRV 18.7UWood/m2, 100% OVT was perform and a decrease in pulmonary pressure and PVR was found (68/19 mmHg, mean of 41 and PVR 7.8UWood/m2). 20 months after surgery, CT (Figure 2 - see full pdf) and catheterization was performed and found a mean pulmonary pressure of 33mmHg and PVR 12Uwoods/m2, the clinical evolution is favorable, it receives sildenafil.

Some authors suggest that surgical correction should be performed during the first three months of life. We report the case of an older patient of all series undergoing surgical correction of AORPA.

About the author

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Susan Pumacayo

Cardiólogo Pediatra

Hospital Nacional Edgardo Rebagliatti Martins


Key Contributors

Susan Pumacayo-Cárdenas, Moisés Jimenez-Santos, Jose Garcia-Montes y Edgar Quea-Pinto. Department of Cardiac Tomography. National Institute of Cardiology Ignacio Chávez. México. Department of interventional cardiology of congenital heart disease. National Institute of Cardiology Ignacio Chávez. México.

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