[Perioperative management of lung transplantations in patients with pulmonary arterial hypertension compared with that in patients with lymphangiomyomatosis].

[lymphangioleiomyomatosis]

The number of lung transplantation has tended to increase as a treatment for patients with pulmonary arterial hypertension (PAH ) and lymphangiomyomatosis (LAM ) in Japan . However , we have little evidence about the comparison of perioperative management in patients with PAH and that in patients with LAM .In this retrospective study , ten patients with PAH and seventeen patients with LAM who underwent the lung transplantations between 2006 and 2011 were enrolled .PAH patients received double lung transplantation with intraoperative cardiopulmonary bypass (CPB ) support . Before anesthesia induction , percutaneous cardiopulmonary support (PCPS ) was begun . Most of LAM patients received single lung transplantation without using CPB support and PCPS support before anesthesia induction . But sometimes during an operation PCPS support was necessary . Postoperative PCPS support showed no significant differences between PAH and LAM . The four year survival rate was 80 % (PAH ) and 87 .8 % (LAM ). Pao 2 /FIo 2 in patients with PAH and in those with LAM , %FEV 10 in those with LAM after surgery improved when compared to those before surgery .The large difference in perioperative management between patients with PAH and those with LAM is an important knowledge for anesthesist .

Diseases
Validation

Diseases presenting "hypertension" symptom

achondroplasia

acute rheumatic fever

adrenal incidentaloma

aniridia

aromatase deficiency

cadasil

child syndrome

cohen syndrome

congenital adrenal hyperplasia

congenital diaphragmatic hernia

cushing syndrome

cystinuria

erdheim-chester disease

erythropoietic protoporphyria

esophageal adenocarcinoma

fabry disease

familial hypocalciuric hypercalcemia

gm1 gangliosidosis

heparin-induced thrombocytopenia

hereditary cerebral hemorrhage with amyloidosis

holt-oram syndrome

homocystinuria without methylmalonic aciduria

hydrocephalus with stenosis of the aqueduct of sylvius

inclusion body myositis

kallmann syndrome

kindler syndrome

lamellar ichthyosis

lymphangioleiomyomatosis

pendred syndrome

primary effusion lymphoma

scrub typhus

severe combined immunodeficiency

sneddon syndrome

typhoid

von hippel-lindau disease

well-differentiated liposarcoma

werner syndrome

x-linked adrenoleukodystrophy

zellweger syndrome

This symptom has already been validated