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Pulmonary arteriopathy (PA) is the pathologic hallmark in human medicine of diffuse constrictive (medial and intimal remodeling) or multifocal complex (plexiform and dilatative lesions) arterial lesions, or both, that lead to irreversible obliteration of the arterial lumen. Clinically, PA leads to pulmonary arterial hypertension (PAH), of which idiopathic (IPAH) is one of the 5 subsets, and ultimately, to right-sided heart failure (RHF). Clinical and pathologic findings from 6 dogs with diagnosis of IPAH and PA were reviewed. These dogs were of various pure (5/6, 83%) and mixed (1/6, 17%) breeding, 5 months to 9 years (mean 5.2 years) old, and predominantly female (4/6, 67%) and reproductively intact (4/6, 67%). Doppler echocardiography (n = 5) indicated increased pulmonary arterial pressures during systole (70-135 mm Hg, mean 98 mm Hg) and diastole (35-80 mm Hg, mean 58 mm Hg). All 6 dogs had right ventricular pressure overload, right ventricular eccentric hypertrophy, and RHF. Histologic examination confirmed the clinical diagnosis of IPAH in all dogs, revealing PA characterized by 1 of the 4 main human histologic subsets: 1) isolated medial hypertrophy (1/6, 17%); 2) medial hypertrophy-intimal thickening without the plexiform lesion (1/6, 17%); 3) medial hypertrophy-intimal thickening concurrent with the plexiform lesion, which often was regionally clustered and situated near branching points of the respiratory artery, the poststenotic dilatation lesion, and vasculitis (4/6, 66%); and 4) isolated arteritis (1/6, 17%). Ancillary lesions similar to those in humans also complicated the PA (5/6, 83%). The complex lesions and ancillary exudative alveolitis seemed to be important indicators of severe, likely rapidly progressive and fatal, IPAH.

At the University of California, San Diego pulmonary thromboendarterectomy (PTE) has emerged as an effective measure in the treatment of chronic thromboembolic pulmonary hypertension. Unresolved emboli become organized by incorporation into the vascular wall and may form strictures, webs, bands and/or membranous occlusions and cause pulmonary hypertension refractory to medical treatment. When pulmonary vascular resistance exceeds 300 dyn.sec.cm-5 and the vascular wall changes are located to begin at or proximal to the lobar artery level, surgery is indicated. The operation is performed using cardiopulmonary bypass, deep hypothermia and periods of circulatory arrest. The dissection of each segmental artery is carried out in the media layer from separate incisions in the right and left pulmonary artery at the level of the pericardial flexion. Pulmonary reperfusion edema may complicates the postoperative course, and pulmonary hemorrhage, respiratory insufficiency necessitating prolonged ventilatory support and secondary multi organ failure are main causes of hospital mortality. Between October 1984 and September 1988 103 patients with a mean age of 50 +/- 16 years underwent PTE. Consequently, pulmonary vascular resistance could be reduced from 788 +/- 370 to 299 +/- 150 dyn.sec.cm-5 and cardiac index increased from 2.0 +/- 0.6 to 3.2 +/- 0.8 l/min-m2. Hospital mortality was 11.7% (12/103 patients). Thus, pulmonary thromboendarterectomy effectively reduces pulmonary hypertension at an acceptable low risk. The results indicate that patients should be diagnose and referred for surgery as early as possible.