title: [Circulatory failure in children with left-to-right shunt in the framework of congenital heart defects: pathophysiology and therapeutic results].
abstract: In children with large left-to-right shunts secondary to congenital heart defects the imbalance between the pulmonary and systemic perfusion may lead to circulatory congestion with clinical signs similar to those of heart failure. The circulatory function in this state was evaluated by using the invasive measurements performed during cardiac catheterisations in n = 64 young patients with ventricular septal defect (n = 56) or complete atrioventricular septal defect (n = 8) aging 0.1-23.7 years (median 1.1 years). The mean shunt ratio was Qp/Qs = 2.4 (range 1-8). With increasing shunt ratio the pulmonary perfusion raised (r = 0.84), but the systemic output dropped significantly (r = -0.77) while the total cardiac output (Qp + Qs) increased slightly not exceeding 141/min/m2. In infants, the systemic hypoperfusion affects the hemoglobin content: Hb = 14.9-1.01 x Qs, r = 0.63, p < 0.01. This may be due to the diminished oxygen extraction reserve of 46%. With dropping systemic output, the vascular resistance increases and the mean aortic pressure (MAP) remains normal. The actual pressure values layed near to the curve of the normal aortic pressure calculated as MAP = Qs x Rs. This pressure-flow-resistance diagram was used to interpret the effects of vasodilators established by 7 studies: ACE-Inhibitors, Hydralazine, and Na-Nitroprusside reduce the vascular resistance effectively but induce hypotension, because the systemic output fails to increase. In the chronic circulatory congestion secondary to a large intracardiac left-to-right shunt the pulmonary perfusion increases with the shunt ratio but the systemic output decreases and the total cardiac output is limited to a maximum of 141/min/m2. In this state vasodilators cause systemic hypotension thus offering no acceptable therapeutic option.
authors.affiliation: Klinik für Pädiatrische Kardiologie, Georg-August-Universität Göttingen.
authors.forename: A
authors.initials: A
authors.lastname: Wessel
authors.forename: R
authors.initials: R
authors.lastname: Buchhorn
authors.forename: J
authors.initials: J
authors.lastname: Bürsch
mesh_terms: D000293:Adolescent; D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D000818:Animals; D002303:Cardiac Output, Low / Q000188:drug therapy / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004285:Dogs; D005260:Female; D006333:Heart Failure / Q000188:drug therapy* / Q000209:etiology*; D006343:Heart Septal Defects / Q000503:physiopathology*; D006344:Heart Septal Defects, Atrial / Q000503:physiopathology; D006345:Heart Septal Defects, Ventricular / Q000503:physiopathology; D006439:Hemodynamics; D006801:Humans; D006830:Hydralazine / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009599:Nitroprusside / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D014665:Vasodilator Agents; D009599:Nitroprusside; D006830:Hydralazine