, we attempted percutaneous transcatheter closure of seven ventricular septal defects (VSD) in six patients; none of the patients was a candidate for operative management. Patients' ages ranged from 8 months to 82 years (6.0-70 kg); diagnoses included postinfarction VSD (n = 4), congenital VSD (n = 1), and postoperative congenital VSD (n = 2). Indications for VSD closure were shock or respiratory failure (n = 5) or multiple episodes of endocarditis (n = 1). Closure was attempted with a Rashkind double umbrella: VSDs were crossed via the left ventricle and a guide wire was advanced to the right heart, snared with a venous catheter, and used to direct a long sheath (and ultimately the double umbrella) across the VSD. We crossed the VSD in all seven attempts, and a 17-mm double umbrella was successfully placed in each VSD. In the first (postinfarction) patient with the largest (12 mm) VSD, the umbrella embolized after 20 seconds to the pulmonary artery (without reducing flow). The other six umbrellas remained in position, either diminishing or abolishing the left-to-right shunts. Postinfarction patients had increasing VSD shunting over the next several days and died; at postmortem, the umbrellas remained well positioned in the septum, with other VSDs present. All three congenital VSDs had absent or diminished shunts after umbrella closure. These preliminary data indicate that transcatheter VSD closure is feasible in selected cases.

We postulate that PAVMs after CVPA are related to the diversion of normal hepatic venous flow from the pulmonary circulation. In this sense, these PAVMs may be analogous to those associated with liver disease, which have been found to resolve after liver transplantation. Redirection of hepatic flow to the pulmonary bed in some patients with CHD and PAVMs may lead to reversibility of the PAVMs.

Background-Transcatheter balloon aortic valvuloplasty (BAVP) has become the first-line treatment for critical aortic stenosis (AS) in neonates. However, little is known about the growth and function of left heart structures or about patterns of reintervention on the left heart after neonatal BAVP. Methods and Results-Between 1985 and 2002, 113 patients underwent neonatal BAVP at Յ60 days of age. There were 16 early deaths (14%), with a significant decrease from 1985 to 1993 (22%) to 1994 to 2002 (4%), and 6 patients had successful early conversion to a univentricular circulation. In the short term, the mean relative gradient reduction was 54Ϯ26%, and significant aortic regurgitation (AR) developed in 15% of patients. The 91 early survivors with a biventricular circulation were followed up for 6.3Ϯ5.3 years, during which time there was a steady increase in the frequency of significant AR. Freedom from moderate or severe AR was 65% at 5 years. In almost all patients with a baseline aortic annulus z score less than Ϫ1, the annulus diameter increased to within the normal range within 1 to 2 years. Similarly, left ventricular (LV) end-diastolic dimension z scores, which ranged from Ϫ5 to 7.5 before BAVP, normalized within 1 to 2 years in nearly all patients with a predilation z score less than Ϫ1. Among early survivors with a biventricular circulation, reintervention-free survival on the LV outflow tract was 65% at 1 year and 48% at 5 years, with younger age, higher pre-and post-BAVP gradients, and a larger balloon-annulus diameter ratio associated with decreased reintervention-free survival (PϽ0.01). Seventeen surgical interventions were performed on the aortic valve in 15 patients, including replacement in 7. Survival free from aortic valve replacement was 84% at 5 years. Conclusions-BAVP for AS during the first 60 days of life results in short-term relief of AS in the majority of patients.Among early survivors, initially small left heart structures may be associated with worse subacute outcomes but typically normalize within 1 year. Reintervention for residual/recurrent AS or iatrogenic AR is relatively common, particularly during the first year after BAVP, but aortic valve replacement during early childhood is seldom necessary.

Aortico-left ventricular tunnel is a rare cardiac malformation with a good post-operative long-term outcome. Associated lesions occurred in 45%. Catheterization should be reserved for patients with unclear non-invasive findings or transcatheter closure. We recommend surgery for most patients. We report spontaneous closure in one patient, prompting consideration of conservative follow-up in rare small, asymptomatic AVLT.

At the time of surgical repair, a lung biopsy was performed on patients with congenital heart defects who either had pulmonary hypertension or in whom it would be likely to develop if the lesion were not corrected. Pulmonary vascular changes, assessed morphometrically and also according to the classification of Heath and Edwards (Circulation 18: 533, 1958), were correlated with the postoperative pulmonary hemodynamic findings: mean pulmonary arterial pressure the day after correction and mean pulmonary arterial pressure and pulmonary vascular resistance measured 1 year later. On the first postoperative day, increased mean pulmonary arterial pressure was uncommon in patients with morphometric grade A or B (mild) biopsy findings and Heath-Edwards grade N (normal), and if it was present it was of a mild degree. Mean pulmonary arterial pressure was commonly elevated in those with grade B (severe) or C (mild or severe) and Heath-Edwards grade biopsy results and was more frequently elevated in those with grade findings. Moderate-to-severe elevation of mean pulmonary arterial pressure was invariable in patients with Heath-Edwards grade III changes regardless of the morphometric grade. One year after repair, mean pulmonary arterial pressure and/or pulmonary vascular resistance were normal in all patients whose conditions were corrected surgically before 9 months of age regardless of the severity of the pulmonary vascular changes. Values were normal in patients whose conditions were repaired surgically at 9 months of age or later who had grade A or B (mild) morphometric findings with any Heath-Edwards grade or grade B (severe) morphometric findings with Heath-Edwards grade I but were increased in half of the patients with grade B (severe) morphometric findings and Heath-Edwards grade II or with grade C (mild or severe) and HeathEdwards grade I or LI changes. Pulmonary arterial pressure and pulmonary vascular resistance were increased in all patients whose conditions were repaired after 2 years of age with grade C morphometric findings and to a severe degree if associated with Heath-Edwards grade III. Thus, although the HeathEdwards grade can usually be used to identify patients at risk for pulmonary hypertension in the early postoperative period, both the morphometric and the Heath-Edwards grades as well as the age of the patient at the time of repair can be used to determine whether pulmonary arterial pressure and resistance eventually return to normal or remain elevated. Circulation 69, No. 4, 655-667, 1984. WE PREVIOUSLY analyzed lung tissue obtained at biopsy during surgical repair from patients with congenital heart defects and correlated the morphometric findings with the preoperative pulmonary hemodynamic data.' We observed abnormalities in muscularization and growth of the pulmonary arteries associated with increased pulmonary blood flow, pressure, and resistance. In the present study, both the morphometric findings and the changes described by Heath and Edwards2 have been correlated with mean pulmona...

The records of 90 patients with Wolff-Parkinson-White syndrome who presented with supraventricular tachycardia in the first 4 months of life were reviewed. Among these, 63% were male. Structural heart disease was present in 20%, most commonly Ebstein's anomaly. All patients presented with a regular narrow QRS tachycardia, and pre-excitation became evident only when normal sinus rhythm was established. Only one infant had atrial flutter and none had atrial fibrillation. Type A Wolff-Parkinson-White syndrome was most common (49%), with heart disease occurring in only 5% of these patients. In contrast, heart disease was identified in 45% of those with type B syndrome. Initially, normal sinus rhythm was achieved in 88% of the 66 infants treated with digoxin with no deaths. Normal sinus rhythm resumed after electrical countershock in 87% of the 15 infants so treated. Maintenance digoxin therapy was used in 85 patients. The Wolff-Parkinson-White pattern disappeared in 36% of the patients. Four infants died of cardiac causes during the mean follow-up period of 6.5 years. Two of these four infants had congenital heart disease; the third, with a normal heart initially, developed ventricular fibrillation and died from a cardiomyopathy considered related to resuscitation. The remaining infant, with a normal heart, died suddenly at 1 month of age. All were receiving digoxin. A wide QRS tachycardia later appeared in three patients, all with heart disease, one of whom died.(ABSTRACT TRUNCATED AT 250 WORDS)

Both of our original scoring systems are less accurate at predicting outcome than in our original analysis. Revised discriminant analysis yielded a model similar to our original equation that was 90% accurate at predicting survival with a biventricular circulation among neonates with AS and a mitral valve area z-score >-2.

Baffle fenestration is associated with low mortality, significantly less pleural effusion, and significantly shorter hospitalization among high-risk patients having a modified Fontan operation.