Hemodynamic Events during the Development of Cyanosis and Heart Failure in a Patient with Large Ventricular Septal Defect.

THE late occurrence of cyanosis or con-gestive heart failure is not unusual in patients with a large ventricular septal defect and hiigh ptulmonary vascullar resistance.' 2 2 The physiological clhanges producing these events remain somexvhat unclear, however, although recent evidence" "I indlicates that pul-moniarv vascular resistance, if high early in life, may increase over a period of years. In two patients reported by Arcilla and co-work-erss,3 xvascular resistance in the lungs rose su-ffi-ciently to cauise right-to-left intracardiae shuinting and cyanosis. llemodynamic alterations dturing the appearan.ce of heart failure lhalv(e not been reported in these or otlher stidies, hx owever-. ' ` The 1)resent report describes ser;ial hemo-dyn-iamic finidings in a patient with this syndrome (luriiinig a period when cyanosis increased and cardiac faillure occturred. Unlike the patienits mentioned above, ouir patient showed a decrease in arterial oxygen satuira-tion that did not result from a disproportioniate rise in pulmonary compared to systemic vasei-lar resistance. Rather, a progressive fall in the amnouniit of blood pumped from both sideN of the heart explained the intensificationi of cyanosis and the manifestations of heart failure. A Negro man-(n.o. 493722), born io Alabaima imi 19:36, x\vas (liscovered to haxe hleart mriormri sho.rtlv after birtlh. Historical details are lacking for the first [ew yeairs of life, b.utt exertionial dysp-nea severe eniotiglh to prevenit him from ruin-niing or playling xith other bhos xas present as early as age 5. When he vas 12, his physician observed cardiac enlargement and a loud murmur , rales in the right lung, and a palpable liver. l3lood pressure xxas 130 70. An x-ray (fig. 1) shoxxed cardliae enlargement and increase.d pul-monarvy vascular mar-kinigs. He xxwas placed oni digitalis at that time. Improvemenit was grl-adual until int the late 1950's he cotuld climb six flights of stairs or play basketball stren-uiouislv for an houir or two xithout more dxvspn-ea thani his cohorts. 1)igitalis iVsaS stoppe(l (InI iLlg that perio(d. He fti-st visited this hiospitaI] in 19159 fIor cx tioni of cardiac mulr-muir. No complalinits wex'r piresent at that time. lie xxas a xxell-devxe1l)pecl slim mani slhoxiii-ig slight evailosis of finigers anl toes , Ioit ni.o eluilnl)iig. The nieck vessels wexee niot remiarkable. A imiildl thoracic scoliosis xx azis ap~ paei0 emt. A rather rliffose, tlouiglb forcefill apex' 1 in)l polse w as xisible in time fiftlt iinte] space 10 cin. to tlhe left of the steri-nm. A slight precorellial bilge xx as piresent. …