Since the Fontan procedure results in low pulsatile pulmonary blood flow similar to that seen in patients with a Glenn shunt, it may also be associated with abnormal distribution of flow to the lower lung lobes and with the development of pulmonary arteriovenous fistulae (PAVF). In 12 patients 0.8 to 4.5 years after Fontan procedure and in 20 patients 0.2 to 18 years after receipt of Glenn shunts we assessed ventilation (with '33Xe) and perfusion (after a peripheral injection of 99mTc-macroaggregated albumin) to compare upper to lower lobe distribution of blood flow with that in a control group. The presence of PAVF was assessed by radionuclide activity in kidneys and the brain and by a twodimensional echocardiographic contrast study. A decreased upper/lower lobe perfusion ratio was noted in 13 of 20 patients with Glenn shunts (65%) and correlated with the time after surgery (p < .05). Despite the shorter follow-up period, two of 12 (16%) patients who had undergone the Fontan procedure also had a decreased upper/lower lobe perfusion ratio, and one of these developed right heart failure. Brain and kidney radionuclide counts above control values were observed in all patients with Glenn shunts and in 11 of 12 patients who had the Fontan operation. However, in only five of 20 (25%) patients with Glenn shunts were PAVF confirmed by the two-dimensional echocardiographic contrast study. Three of the five patients with PAVF had Glenn shunts of long duration. While only two of five patients with PAVF had a decreased upper/lower lobe perfusion ratio at the time of the study, this abnormality may have been present in the other three at an earlier stage. Our data suggest that a decreased ratio of upper/lower lobe perfusion may be one of several factors associated with the development of PAVF after a Glenn shunt. Also, longer follow-up of patients who have had a Fontan procedure will be necessary to determine whether they are also a group at risk. Circulation 72, No. 3, 471-479, 1985 We therefore performed ventilation/perfusion lung scans to determine the distribution of pulmonary blood flow in patients after the Fontan procedure (RA-PA and RA-RV). We assessed the presence of PAVF by the scintigraphic appearance of 99mTc-macroaggregated albumin (MAA) in the brain and kidneys after a peripheral intravenous injection and by two-dimensional echocardiographic contrast studies. Our findings were compared with those obtained in patients with Glenn shunts and in normal subjects.
Early postoperative atrial tachyarrhythmias, length of follow-up and atriopulmonary connection are significant independent risk factors for the presence of late atrial tachyarrhythmias.
Subaortic stenosis is well known to complicate the clinical course of patients with single ventricle or univentricular hearts, and we have previously suggested that the development of subaortic stenosis in such patients may be causal to and/or accelerated by previous banding of the main pulmonary trunk. To further define the relationship between banding of the pulmonary artery in patients with univentricular hearts and the development of subaortic stenosis, we examined the morphologic substrate and timing of the development of subaortic stenosis in 43 patients seen at our institution from January 1, 1970, through June 30, 1985. These 43 patients include all patients in this period with an unequivocal univentricular heart whose longitudinal data was available for follow-up. We excluded patients who died within 1 week of surgery, patients lost to follow-up, and patients with evidence of subaortic stenosis before banding. Thirty-one of 43 patients (72. 1 %) developed subaortic stenosis subsequent to banding of the main pulmonary artery. The mean age at banding of those patients who developed subaortic stenosis was 0.21 years, and subaortic stenosis was recognized at a mean age of 2.52 years. For the specific cohort of patients whose ventricular morphology was a main chamber of left ventricular type supporting the pulmonary artery and a rudimentary right ventricle supporting the transposed aorta (32 patients), 27 developed subaortic stenosis (84.4%). Subaortic stenosis in the classic form of single ventricle usually results from progressive restriction of a wholly muscular interventricular communication. Banding of the pulmonary artery by producing myocardial hypertrophy undoubtedly accelerates the potential for subaortic stenosis in these patients. Furthermore, one must realize that subaortic stenosis may be present in the absence of a resting pressure gradient, and such subaortic stenosis can usually be unmasked by stimulation with isoprenaline. Finally, one must be guarded in advocating banding of the pulmonary artery in patients with single ventricle, realizing that subaortic stenosis strongly influences the outcome of more definitive surgery in these patients. Circulation 73, No. 4, 758-764, 1986. THE NATURAL HISTORY of patients with single ventricle (one-ventricle hearts, univentricular hearts, univentricular atrioventricular connection) has been irrevocably altered by those diverse surgical procedures that (1) augment pulmonary blood flow, (2) reduce pulmonary blood flow, (3) augment atrial mixing when intracardiac streaming is disadvantageous, or (4)
The medical and radiological records of 64 consecutive infants and children who underwent transfemoral balloon dilation of the aorta or aortic valve were reviewed to determine the incidence, nature, and post-treatment outcome of acute iliofemoral complications. 29), and magnetic resonance imaging (three of 29). Of eight patients, three with arterial disruption had acute hypotension requiring transfusion and immediate surgery; the other five had absent pedal pulses after the procedure. Of these five, three developed bleeding during thrombolytic therapy and underwent surgical exploration, and two were diagnosed by angiography after ineffective thrombolytic therapy. Angiography in three patients with iliac artery avulsion showed tapered occlusion in two and an aneurysm in one. In patients with iliofemoral thrombosis, angiography showed occlusion from the puncture site to the origin of the external iliac artery. Eleven patients (17% of the entire group and 38% of the group with acute iliofemoral complications) had reduced or absent pedal pulses at the time of discharge. A significant correlation was found between increased incidence of iliofemoral thrombosis and disruption (as well as abnormal pedal pulses at hospital discharge) and low patient weight. (Circulation 1990;82:1697-1704 Cmomplications involving the iliac and femoral arteries occur relatively frequently after diagnostic catheter procedures in infants and children, especially in those weighing less than 15 kg.1-6 Long-term sequelae of iliofemoral thrombosis, including lower-extremity growth disturbance, have resulted in some patients, although attempts to determine the incidence of such complications have yielded varying results.7-15 To avoid these complica-
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.