Abstract
Primary pulmonary hypertension (PPH) is an uncommon disease of unknown causes. The clinical diagnosis requires the demonstration of an elevated pulmonary artery pressure in the absence of known causes or other lung and heart disease. Many studies have reported that PPH is a progressive disease, with an average survival period ranging 2-3 years after diagnosis. A 39-year-old man, who was diagnosed as PPH four years ago, was admitted because of exertional shortness of breath and a mild chest pain. Right and left heart catheterization with coronary arteriography were performed. Pulmonary arterial pressure was elevated (84/32mmHg, mean 49), without any shunt or valvular disease. Coronary arteriogram revealed large and tortuous vessels arising from the atrial circumflex branch to left bronchial artery, and from the sinus node branch to right bronchial artery.
Anastomosis between the coronary and bronchial arteries are known to exist but very rare especially in adult. Large anastomosis like this case may be congenital anomalies of coronary artery. The shunting of blood caused the coronary steal syndrome resulting in angina-like chest pain. We report here a case of PPH with rare abnormalities, in which both bronchial arteries arising from coronary arteries, and the relationship between PPH and anastomosis of coronary artery to bronchial artery is discussed.
