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Background: Experience of balloon dilation of peripheral pulmonary stenosis (PPS) in Williams syndrome (WS) is limited., Methods and Results: Catheterizations in all patients with WS undergoing therapy for PPS from 1984 to 1999 were reviewed. Criteria for successful dilation included an increase >50% in predilation diameter and a decrease >20% in ratio of right ventricular (RV) to aortic (Ao) systolic pressure. Median age and weight were 1.5 years and 9.5 kg. There were 134 dilations during 39 procedures in 25 patients. The success rate for initial dilations was 51%. In multivariate analysis, successful dilation was more likely (1) in distal than in central pulmonary arteries (P=0.02), (2) if the balloon waist resolved with inflation (P=0.001), and (3) with larger balloon/stenosis ratio (P<0.001). RV pressure was unchanged after dilation (96+/-30 versus 97+/-31 mm Hg), primarily because of failure to enlarge central pulmonary arteries. The Ao pressure increased (102+/-14 versus 109+/-19 mm Hg, P=0.03), and the RV/Ao pressure ratio decreased (0.97+/-0.34 versus 0.91+/-0.30, P=0.05). Aneurysms developed after 24 dilations (18%) and were not related to balloon/stenosis ratio. Balloon rupture in 12 dilations produced an aneurysm in all 7 cases when rupture was in a hypoplastic segment. Three patients died, none from pulmonary artery trauma, and all before 1994., Conclusions: Mortality occurred early in our experience. Despite successful dilation of distal pulmonary arteries, there was modest initial hemodynamic improvement, mainly because of persistent central pulmonary artery obstruction. A serial approach of distal dilations followed by surgical repair of proximal obstruction may be a rational and successful therapy.

Background: Effective transcatheter or surgical closure of apical muscular ventricular septal defects (VSDs) requires accurate delineation of variable and often complex anatomy. These defects have generally been considered as communications between the apexes of both left and right ventricles., Methods and Results: Among 50 consecutive patients with multiple muscular VSDs referred for transcatheter device closure between October 1987 and April 1993, a subset of 10 patients (aged 7 days to 28 years) with apical muscular VSDs shared a unique set of anatomic characteristics: (1) large and often single opening in the left ventricle; (2) multiple right ventricular openings in the anterior aspect of the apical septum; and (3) separation of the right ventricular apical region into which the VSDs open from the rest of the right ventricular inflow and outflow by prominent muscle bundles. Additional analysis of the anatomy by use of echocardiography and cineangiography showed that these muscular defects were between the left ventricular apex and right ventricular infundibular apex. In 6 patients, the transcatheter devices used to create a septum in these hearts were placed in the right ventricle, straddling muscle bundles that separated the apical VSD from the rest of the right ventricular inflow and outflow, resulting in incorporation of a portion of the right ventricular infundibular apex into the physiological left ventricle. Three patients had devices placed between the apexes of the left ventricle and the infundibulum. The defect closed spontaneously within the right ventricle in 1 patient. One patient died after surgery for tetralogy of Fallot in situs inversus. The remaining 9 patients were all clinically well at the time of their most recent follow-up visit (follow-up duration, 32 +/- 11 months). This distinct type of apical VSD was identified by echocardiography in 20 of 274 patients who were followed up clinically for muscular VSDs., Conclusions: Left ventricular-infundibular apical VSDs constitute a distinct morphological type of muscular VSD that can be distinguished by echocardiography and cineangiography. In selected cases, the infundibular apex can be separated from the rest of the right ventricular inflow and outflow to eliminate flow across these defects.

Background: Previous reports suggest a poor prognosis for patients with ectopia cordis and significant congenital heart disease., Methods and Results: To determine the outcome of affected patients in a recent medical and surgical era, we reviewed the clinical course, echocardiograms, catheterization data, and operative reports of all patients with ectopia cordis and significant heart disease encountered at Children's Hospital from 1982 to the present. We identified 13 patients with conotruncal defects and either thoracic (TEC, n = 4) or thoracoabdominal (TAEC, n = 9) ectopia cordis. Diagnosis was established in utero in 6, with termination of pregnancy in 2 and death before transfer to Children's Hospital in 1. Of 10 patients postnatally managed at Children's Hospital, 4 of 8 with TAEC and 1 of 2 with TEC survived beyond infancy, with an age at most recent follow-up of 3.5 to 9.8 years. All 5 survivors had procedures as neonates to provide skin coverage over the midline defect and heart, with concomitant repair of a small omphalocele in 4. Three underwent successful surgical repair of the cardiac defect, including ventricular septal defect closure for double-outlet right ventricle (9 months), repair of tetralogy of Fallot with pulmonary atresia (2.6 years), and repair of double-outlet right ventricle, pulmonary stenosis, and total anomalous pulmonary venous connection to the coronary sinus (3.3 years). Two other patients with TAEC, conoventricular defects, and hypoplastic left ventricle underwent single-ventricle palliation. One patient, at 4 years of age underwent a right cavopulmonary anastomosis and intra-atrial coronary sinus-to-right superior vena cava baffle. The second patient had a modified Fontan procedure. None of the 5 survivors had significant extrathoracic defects, whereas all 3 who died by 3 weeks of age had both a large omphalocele and pulmonary hypoplasia. Two infants with pentalogy of Cantrell, 1 with a large omphalocele, died of fungal sepsis by 5 weeks of age, and 1 other died intraoperatively after repair of pulmonary venous confluence obstruction and right Blalock-Taussig shunt placement., Conclusions: Our experience indicates that patients with TEC and TAEC and significant intracardiac defects, in the absence of significant extracardiac defects, can survive beyond early infancy and undergo successful cardiac repair or more definitive palliation for single-ventricle physiology.

Background: Isolated peripheral pulmonary artery stenosis (PPS) in the adult is rare and frequently unsuspected. We review in this article our experience with 12 adult patients with isolated PPS, half of whom had been previously diagnosed with chronic pulmonary thromboembolic disease., Methods and Results: The presentation, evolution, and management of 12 adults with isolated PPS, 17 to 51 years of age (mean, 36.2 +/- 9.7 years), were evaluated. Presenting symptoms were dyspnea and fatigue. Three patients had New York Heart Association (NYHA) functional class III or greater. Lung perfusion scans revealed multiple segmental abnormalities in flow distribution in all patients. Oxygen desaturation at rest was present in 4 patients. At catheterization, right ventricular (RV) pressure was suprasystemic in 2 patients, systemic in 1, and more than half-systemic in 7. All had multiple bilateral non-uniform stenoses in segmental and subsegmental arteries. Balloon pulmonary angioplasty (BPA) to decrease RV hypertension and improve pulmonary flow distribution was performed in 11 patients. After BPA, vessel diameter increased > 50% in 10 patients, distal pulmonary artery pressure increased > or = 30% in 6, and RV pressure decreased > 30% in 5. One patient died shortly after BPA as a result of pulmonary hemorrhage. Immediate procedural success was achieved in 9 of 11 patients. At a mean follow-up period of 52 +/- 32 months, 7 patients had sustained symptomatic improvement (NYHA class I-II)., Conclusions: We describe a severe syndrome of isolated PPS in the adult that mimics chronic pulmonary thromboembolic disease. Pulmonary hemodynamics and angiography are required for definitive diagnosis. BPA may offer these patients successful short-term reduction in RV hypertension and alleviation of symptomatology.

Background: Right ventricle-to-pulmonary artery (RV-PA) homografts and bioprosthetic conduits are commonly used to palliate various types of complex congenital heart disease. These conduits frequently develop progressive obstruction and require surgical replacement. This report reviews our experience implanting balloon-expandable stents to relieve conduit obstruction and delay reoperation., Methods and Results: A retrospective review identified 44 patients who underwent placement of 48 stents in obstructed RV-PA conduits. Median patient age was 6.9 years (range, 7 months to 30 years), and median follow-up time was 14.2 months (range, 0 to 48 months). Stent implantation initially decreased the RV-PA pressure gradient from 61.0 +/- 16.9 to 29.7 +/- 11.9 mm Hg (P < or = .001) and the right ventricular-to-systemic arterial pressure ratio from 0.92 +/- 0.17 to 0.63 +/- 0.20 (P < or = .001). The diameter of the stenotic region expanded from 9.3 +/- 3.5 to 12.3 +/- 3.3 mm in the anteroposterior view (P < or = .001) and from 6.6 +/- 2.9 to 10.9 +/- 2.5 mm in the lateral view (P < or = .001). During the follow-up period, 2 patients had their stents redilated, 7 had additional conduit stents deployed, and 14 underwent surgical replacement of their conduits. Actuarial freedom from conduit reoperation was 65% at 30 months postprocedure. Seven patients were found to have fractured stents on follow-up, suggesting an important role for external compressive forces in conduit failure. Recatheterization in 16 patients a median of 11.8 months (3 to 48 months) postprocedure demonstrated hemodynamic evidence of recurrent obstruction despite sustained enlargement at the previously stented sites. Complications included stent displacement (n = 1), bacterial endocarditis (n = 1), and false aneurysm formation (n = 1). One patient died awaiting conduit replacement surgery., Conclusions: Stent implantation in obstructed RV-PA conduits results in significant immediate hemodynamic and angiographic improvement. In a subgroup of patients, the procedure prolongs conduit life span by several years and increases the interval between conduit reoperations. Recurrent obstruction is caused by external compression and progressive stenosis outside the stented region.

Background: Pulmonary arteriovenous malformations (PAVMs) are a known complication after some types of cavopulmonary anastomoses (CVPAs). Their cause is unknown, but they may be related to the absence of pulsatile flow or the presence or absence of circulating factors. These PAVMs are diffuse and are presumed to be progressive and irreversible., Methods and Results: All patients with congenital heart disease (CHD) seen at Children's Hospital, Boston, Mass, between 1970 and 1993 were reviewed. We report on the 10 patients with CHD who were found to have developed PAVMs, as diagnosed by cardiac catheterization. Diagnoses included heterotaxy syndrome/polysplenia, with interrupted inferior vena cava and hepatic veins draining to the right atrium (n = 6); heterotaxy/asplenia (n = 1); corrected transposition with pulmonary stenosis (n = 1); and biliary atresia and associated CHD (n = 2). PAVMs were diagnosed 0.1 to 7.0 years (median, 3.5 years) after creation of a CVPA that resulted in exclusion of hepatic venous flow from one or both lungs in 8 of the 10 patients; the remaining 2 patients had normal drainage of hepatic veins to the lungs but had biliary atresia. In all, the common anatomic feature was the exclusion of normal hepatic venous return from the affected pulmonary arterial circulation. All patients with interrupted inferior vena cava, azygous continuation to the superior vena cava, and hepatic veins draining to the right atrium (polysplenia syndrome) were reviewed to determine the incidence of PAVMs in those with CVPA (ie, hepatic venous flow excluded from the pulmonary arteries) and without CVPA. Six of 28 (21%) of those with versus 1 of 56 (1.8%) of those without CVPA developed PAVMs (P = .004). The 1 patient without CVPA who had PAVMs also had biliary atresia. Among patients with CVPA, the probability of developing PAVMs was 15% and 28% at 3 and 5 years, respectively, after CVPA. The histological and angiographic appearances of PAVMs after CVPA are similar to those seen in PAVMs associated with hepatic cirrhosis., Conclusions: 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: Despite the recent wide applicability of the bidirectional cavopulmonary shunt, there is limited reported experience in performing these shunts in infants 6 months or younger., Methods and Results: Before October 1992, 17 consecutive infants aged 4.2 to 6.5 months (median, 6.1 months) underwent bidirectional cavopulmonary shunts. The diagnoses were hypoplastic left heart syndrome (n = 7), single right ventricle (n = 5), and single left ventricle (n = 5). All but 2 patients had prior palliative surgery. The bidirectional cavopulmonary shunt was performed early on an elective basis in 9 patients; the remaining patients had progressive cyanosis (6 patients), severe ventricular failure (1 patient), and coexisting restrictive bulboventricular foramen (1 patient). The median preoperative pulmonary arterial pressure and pulmonary vascular resistance were 15 mm Hg and 2.3 U.m2, respectively. One patient died; the overall hospital survival was 94%. The most common postoperative problem was transient systemic hypertension, observed in 14 (88%) of 16 survivors. Systemic arterial oxygen saturation increased from a median of 75% before surgery to a median of 85% after surgery (P < .05). The median hospital stay was 6 days. There were no late deaths during follow-up (median, 12.4 months). At postoperative cardiac catheterization performed in 9 of 16 survivors, there was no evidence of severe hypoxemia, shunt narrowing, or pulmonary arteriovenous fistulas. Of the 16 survivors, 6 have had a subsequent Fontan operation at a median age of 1.9 years; there were 5 survivors., Conclusions: Early bidirectional cavopulmonary shunt in young infants has shown encouraging early results and provides improved oxygenation with low morbidity and mortality. We speculate that an early bidirectional cavopulmonary shunt on an elective basis may reduce the deleterious sequelae of chronic hypoxemia, long-term ventricular volume overload, and repeated palliative procedures, thus yielding a more suitable Fontan candidate.

Background: Surgical repair of muscular ventricular septal defects (VSDs) has been associated with significant morbidity and mortality when the defects are multiple, associated with complex cardiac lesions, or requiring left ventriculotomy. Transcatheter VSD closure may be difficult in patients weighing less than 7 kg or those with limited vascular access. We attempted intraoperative device closure of muscular VSDs during surgical repair of associated lesions., Methods and Results: We considered intraoperative umbrella closure in 10 patients, 8 with additional complex heart lesions. "Swiss cheese" defects were present in 4. Median patient age was 4.7 months. Device closure was not attempted in 1 patient due to an inadequate septal rim. Nine umbrellas, 12 to 40 mm in diameter, were positioned to straddle the septum in all 9 patients. There were 3 early deaths, 2 in patients who were moribund preoperatively. The third was due to severe ventricular dysfunction. Among the 6 survivors, 3 had a Qp:Qs < or = 2 and right ventricular or pulmonary artery pressure less than half systemic. These patients had no further intervention. The other 3 had a Qp:Qs > 2. One died unexpectedly 5 weeks after cardiac surgery. The other 2 had subsequent closure of residual VSDs. All 5 survivors are well at follow-up of 8 to 25 months., Conclusions: Intraoperative umbrella VSD closure appears less successful than VSD closure in the catheterization laboratory. In selected patients, particularly very small infants, intraoperative device placement may be advantageous. Completeness of closure is difficult to assess intraoperatively.

Background: Patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries are a high-risk group for whom there is no consensus on the correct approach to medical management. The purpose of this report is to review a 14-year experience in the treatment of these patients comparing management schemes., Methods and Results: Between January 1978 and August 1988, 91 patients with tetralogy of Fallot and pulmonary atresia had an adequate evaluation of their pulmonary artery anatomy before any surgical management. Forty-eight of these patients had diminutive pulmonary arteries (38 to 104 mm2/m2) supplied by aortopulmonary collaterals and were managed in four different fashions. Of the 9 patients repaired primarily, 7 died early and the two survivors had poor hemodynamic outcome. Of the 9 patients conservatively managed with no intervention before 5 years of age, 4 died and only 1 had a satisfactory hemodynamic result after repair. Of 10 shunted patients, 3 died and 3 had satisfactory repairs. Since 1984, we have rehabilitated pulmonary arteries with (1) right ventricle to pulmonary artery surgical graft, (2) balloon dilation of residual pulmonary artery stenoses and embolization of collaterals, and (3) surgical closure of ventricular septal defect and repair of remaining obstructions. Of 20 patients so managed, 7 died after various stages, but 10 of 20 had complete repairs. All repaired patients with subsystemic right ventricular pressures had at least one successful pulmonary artery dilation., Conclusions: A combined catheter-surgery approach begun at an early age in patients with tetralogy of Fallot and pulmonary atresia with diminutive pulmonary arteries appears to enhance the chances of satisfactory complete repair.

Background: Choreoathetosis (CHO) after congenital heart surgery has been described since 1960. Risk factors and patient outcome have not been well defined., Methods and Results: As our complexity of cases increased and management of pH on cardiopulmonary bypass (CPB) evolved, we noted the appearance of CHO among patients beginning in 1986. We reviewed the hospital course and follow-up of all 19 affected children, including eight younger patients (median age, 4.3 months) who developed a mild transient form of CHO, all of whom survived and had complete resolution of CHO; seven of these eight patients had deep hypothermic circulatory arrest (DHCA). Eleven older patients (median age, 16.8 months) developed severe persistent CHO; 11 had DHCA, 10 were cyanotic, seven had anatomic pulmonary atresia, and three others were physiologically analogous with a systemic to pulmonary artery shunt-dependent circulation. Five of six patients who had pertinent preoperative angiography had systemic to pulmonary collateral vessels arising from the head and neck arteries. Mortality in severe patients was 36% (four of 11); the seven survivors showed improvement, but only one had a normal neurological examination after 60 months of follow-up. When severe CHO patients were compared with 17 age- and diagnosis-matched patients without neurological complications, no differences were found in CPB or DHCA times, arterial blood gases, or hematocrits. Time from onset of CPB to onset of DHCA (time to shutoff) was shorter in the severe persistent CHO group than for comparison patients (22 +/- 7 versus 40 +/- 29 minutes, p = 0.053)., Conclusions: Factors that may be associated with the development of severe persistent CHO include 1) age beyond early infancy, 2) cyanotic heart disease with systemic to pulmonary collaterals, particularly those arising from the head and neck vessels, and 3) the duration of the cooling period used in conjunction with deep hypothermic circulatory arrest. We advocate earlier reparative surgery, precise preoperative diagnosis and preoperative or intraoperative control of systemic to pulmonary artery collaterals, and further study of pH effects during CPB on development of CHO.

We investigated whether or not a systemic arteriopulmonary shunt (SAPS) after 1 year of age affects a child's chances for a successful Fontan procedure. From 1967 to 1986, 50 patients more than 1 year old with tricuspid atresia or other complex lesions amenable to a Fontan operation received a SAPS. Follow-up was incomplete in six patients. Two patients died within 1 week of surgery, and two died within 9 months for a total mortality of 8%. Each of the 40 remaining patients was catheterized 6 months to 10.8 years (mean 4.9 years) after placement of a SAPS. By the criteria of Choussat and Fontan, there were one or more adverse SAPS-induced changes in nine patients (23%). Three developed pulmonary hypertension, one with associated elevation of pulmonary arteriolar resistance and pulmonary artery distortion, and one in conjunction with severe congestive heart failure. Six additional patients developed isolated distortion of the ipsilateral pulmonary artery. Of these nine patients, three had successful Fontan operations (33%), two died after the Fontan operation, and four are no longer candidates. In contrast, of 31 patients without documented SAPS-induced complications, 27 have undergone Fontan operations, 23 successfully (74%). Palliation with a SAPS after 1 year of age can place children at increased risk for a subsequent Fontan operation.

Twenty-two consecutive infants and children underwent aortopulmonary shunt operations with polytetrafluoroethylene grafts. Thirteen infants younger than 6 months of age (group I) received 14 grafts (4--5 mm in diameter, 0.5--2.5 cm long). Two shunts became obstructed, including kinking in the single 2.5-cm graft. Eight of the nine long-term survivors had pulmonary artery pressures measured a mean of 14 months postoperatively. In all infants, pulmonary resistance was normal and mean pulmonary artery pressure was less than or equal to 20 mm Hg, except in the infant with a 5-mm graft. In nine children 7 months to 15 years of age (group 2), the rate of graft occlusion was higher (five of nine). There was no evidence of pulmonary artery distortion secondary to shunt placement in either group. On late follow-up, blood hemoglobins were less than 20 g% as late as 30 months of age; acceptable palliation may be expected until at least 2 years of age and 12 kg in weight are reached. We conclude that short, 4-mm-diameter polytetrafluoroethylene grafts provide good palliation in infants with cyanotic congenital heart disease, with minimal risks from pulmonary hypertension or pulmonary artery distortion. Such grafts are less successful in older children, and should only be considered when other forms of surgical relief are not feasible.

Balloon dilation angioplasty (BDA) was attempted nine times in eight infants and children with aortic coarctation. In three infants (all with associated ventricular septal defect or atrioventricular canal and marked hemodynamic instability) dilation was attempted at a site of aortic narrowing that had not been operated on previously. Although the coarctation gradient fell 40% or more over the short term in two of the three, there was no angiographic or late gradient evidence of improvement. All three underwent subsequent coarctation surgery. Five dilations were performed in four infants and children who had previously undergone coarctation surgery (end-to-end anastomosis, attempted jump graft, and subclavian flap) and had residual gradients. Dilation was successful in all five cases, resulting in an increase in the diameter at the coarctation site (4.7 +/- 2.6 to 7.7 +/- 4.0 mm, p less than .05) and a decrease in the gradient measured 24 hr after dilation (42.0 +/- 15.5 to 11.8 +/- 11.2 mm Hg, p less than .05). In one child with a long area of hypoplasia of the thoracic aorta and similar lesions of the brachiocephalic vessels, a preliminary attempt to dilate a severely narrowed subclavian artery was unsuccessful. Postdilation angiography demonstrated evidence of intimal tears in three of five successful dilations. Follow-up (1 to 6 months) has demonstrated continued gradient relief in four of five children. BDA is frequently, but not always, a successful treatment for human aortic coarctation. The chief determinant of success appears to be the nature of the lesion; short-term changes in coarctation gradient are unreliable indicators of success of failure. Although BDA was not associated with mortality or significant morbidity in this group of patients, its role in the management of children with coarctation is yet to be determined.