Early results and follow-up of balloon angioplasty for branch pulmonary artery stenoses.

Two hundred eighteen balloon angioplasty procedures were performed in 135 patients with branch pulmonary artery stenoses from June 1984 to February 1989. Arteries were dilated in patients with tetralogy of Fallot (n = 49), tetralogy of Fallot/pulmonary atresia (n = 64), isolated peripheral pulmonary artery stenoses (n = 58) and "other" lesions (the majority had truncus arteriosus or single ventricle and surgically induced pulmonary artery stenoses (n = 47). Mean age at dilation was 6.6 +/- 6.3 years (range 1 month to 38.5 years). The mean diameter of the lesion increased from 3.8 +/- 1.7 to 5.5 +/- 2.1 mm with dilation (p = 0.001). The overall success rate was 58% (127 of 218 dilations), assessed by the following criteria: an increase greater than or equal to 50% of predilation diameter, an increase greater than 20% in flow to the affected lung or a decrease greater than 20% in systolic right ventricular to aortic pressure ratio. Success did not correlate with patient age. Mean balloon to artery ratio was higher in successful (4.2) than in failed (3.0) angioplasty procedures (p = 0.0001). There were four early deaths: two of the patients had pulmonary artery rupture with angioplasty performed less than 1 month after pulmonary artery surgery. An aneurysm occurred in 11 arteries and transient pulmonary edema in four patients. At angiography performed a mean of 10 months (range 1 to 54) after dilation, the mean diameter of 57 arteries was unchanged (5.5 versus 5.4 mm). However, 5 of 32 initially successfully dilated vessels had returned to predilation size as a result of restenosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Balloon dilation of postoperative right ventricular outflow obstructions.

Balloon dilation was attempted in 16 patients, aged 5 months to 19.5 years, with right ventricular outflow obstruction after repair of congenital heart defects. Stenosis of a valved conduit between the pulmonary ventricle and pulmonary artery was present in nine patients with a mean transvalvular peak systolic ejection gradient of 61.6 +/- 21.0 mm Hg and a mean right ventricle to aorta pressure ratio of 0.9 +/- 0.2. Supravalvular pulmonary stenosis was present in seven patients; in five, stenosis was at the anastomotic site after the arterial switch operation with a mean peak systolic ejection gradient of 72.2 +/- 10.6 mm Hg and mean right ventricle to aorta pressure ratio of 0.93 +/- 0.05. The other two patients had stenosis at a previous pulmonary artery band site with a peak systolic ejection gradient of 60 and 65 mm Hg and right ventricle to aorta pressure ratio of 0.75 and 0.72, respectively. Balloon dilation was successful in three of nine patients with a valved conduit; two of them had additional successful balloon dilation of the right pulmonary artery. In five of the nine patients (including one with successful dilation) the conduit was replaced 5.7 +/- 4.5 months after balloon dilation. Balloon dilation was successful in only one of the five patients with supravalvular pulmonary stenosis after the arterial switch operation and partially successful in the two patients with supravalvular pulmonary stenosis at a previous band site. The success rate of balloon dilation of postoperative right ventricular outflow obstruction is much lower than that for other right heart obstructions.

High pressure balloon angioplasty for branch pulmonary artery stenosis: early experience.

OBJECTIVES: The aim of this study was to evaluate the efficacy and safety of high pressure balloons (17 to 20 atm, Blue Max, Meditech) to dilate branch pulmonary artery stenosis. BACKGROUND: The low success rate (50% to 60%) for angioplasty of branch pulmonary artery stenosis using low pressure balloons is due primarily to the inability to eliminate the balloon waist. Hence, higher inflation pressures may improve results. METHODS: Hemodynamic and angiographic data from 52 patients (0.3 to 34.8 years old) who underwent high pressure balloon dilation of branch pulmonary artery stenosis between October 1990 and February 1992 were reviewed retrospectively, as were data from previous low pressure dilations in these patients. Common diagnoses included tetralogy of Fallot (n = 9), tetralogy of Fallot with pulmonary atresia (n = 23), single ventricle (n = 8) and isolated congenital pulmonary stenosis (n = 7). The 52 patients had 72 vessels dilated. Criteria for success were a > or = 50% increase in vessel diameter or a > 20% decrease in right ventricular to aortic pressure ratio. RESULTS: Of 36 vessels with previously unsuccessful low pressure balloon dilation, 23 (63%) were successfully dilated with high pressure balloons. Of the 36 remaining vessels, 29 (81%) were successfully dilated with high pressure balloons. Factors associated with success were stenosis at a surgical anastomosis and disappearance of the balloon waist with dilation. Aneurysms developed in three vessels. Complications occurred in seven patients (13%); in two patients the distal pulmonary artery was perforated by a stiff guide wire, causing death in one. CONCLUSIONS: Dilation of stenotic peripheral pulmonary arteries with high pressure balloons improves the success rate of angioplasty both in patients who have had unsuccessful dilation with a low pressure balloon and in those without previous attempted dilation.

Transcatheter closure of coronary artery fistulas.

Transcatheter closure of a coronary artery fistula was undertaken in nine patients. There were three fistulas from the left circumflex coronary artery to the coronary sinus, three from the left anterior descending coronary artery to the right ventricular apex, two from the right coronary artery to the superior vena cava/right atrial junction and one fistula from the left circumflex artery to the pulmonary artery. The fistula was closed with Gianturco coils in six patients, a double-umbrella device in two and a combination of an umbrella and coils in one patient. All fistulas are completely occluded. Complications consisted of migration of two coils, one of which was retrieved, and a transient junctional tachycardia in one patient. In an additional three patients with multiple coronary artery fistulas, transcatheter occlusion was not attempted.

Transesophageal echocardiographic guidance of transcatheter ventricular septal defect closure.

OBJECTIVES: This report describes transesophageal echocardiographic guidance of transcatheter closure of ventricular septal defects and its value as an adjunct to fluoroscopy and angiography in this procedure. BACKGROUND: Experience with transcatheter closure of ventricular septal defects has identified a diverse group of patients in whom it may be the procedure of choice. Although facilitating other interventional procedures, such as transcatheter closure of atrial septal defects, the value of transesophageal echocardiographic guidance for transcatheter ventricular septal defect closure has not been documented. METHODS: All patients who underwent ventricular septal defect closure with transesophageal echocardiographic guidance before November 1992 were included. Angiograms and echocardiograms were reviewed to evaluate device position and relation to valve tissue during placement and to assess residual flow after device implantation. The ability of transesophageal echocardiography to assess these variables was compared with fluoroscopy and angiography. RESULTS: Transesophageal echocardiographic guidance was used in 31 of the 83 catheterizations involving transcatheter ventricular septal defect closure performed between February 1990 and November 1992. Under transesophageal echocardiographic guidance, 45 devices were implanted: 23 in muscular ventricular septal defects, 17 in residual postoperative patch margin defects and 5 in other ventricular septal defects. Transesophageal echocardiographic guidance enhanced assessment of device position and proximity to valve structures and markedly improved assessment of residual flow. Assessment of residual flow with transesophageal echocardiography eliminated the need for multiple angiograms in some patients. Combining transesophageal echocardiography with fluoroscopy and angiography provided the most information. CONCLUSIONS: Transesophageal echocardiography facilitates transcatheter closure of ventricular septal defects by improving assessment of device position and effectiveness of closure. It is indicated when device placement is likely to be difficult or may interfere with valve structures or when multiple interventional procedures are anticipated.

Transcatheter closure of a large patent ductus arteriosus with the clamshell septal umbrella.

In 14 patients undergoing transcatheter closure of a large (greater than 4 mm diameter) patent ductus arteriosus, occlusion was attempted with use of the Bard Clamshell septal umbrella. Patient age ranged from 0.7 to 30.4 years. Isolated patent ductus arteriosus was present in 11 patients; 3 had additional congenital heart lesions. Moderate or severe pulmonary hypertension was present in four patients. The diameter of the patent ductus arteriosus ranged from 4.5 to 14 mm, as determined by contrast injection through an 11F sheath or by balloon sizing; it appeared larger by this method than by the standard angiographic method. All 14 patent ductus arteriosi were successfully closed. Prior embolization of a Rashkind umbrella was the reason for using a Clamshell device in three patients; one additional embolization of a Clamshell device occurred. All errant devices were retrieved at cardiac catheterization, without associated hemodynamic instability. No other complications occurred. Among the 14 patients, 11 had complete ductal closure by Doppler color flow mapping at last follow-up and 3 had trivial residual flow. All four patients having associated complex lesions or pulmonary hypertension, or both, had symptomatic improvement after the procedure, although one child (with Shone's anomaly) died 3 months later. The Clamshell device provides stable and effective closure of a large patent ductus arteriosus, and allows transcatheter closure to be offered to some patients who were previously considered unsuitable for this procedure.

Enlarged bronchial arteries after early repair of transposition of the great arteries.

OBJECTIVES: This study was undertaken to define the incidence of enlarged bronchial arteries after early surgical repair of transposition of the great arteries by the arterial switch operation, and to report the results of catheter-directed therapy in five patients. BACKGROUND: Pathologic and angiographic studies have demonstrated enlarged bronchial arteries in patients with transposition of the great arteries. METHODS: A subjective 4-point scale was used to grade postoperative angiograms performed in 119 patients at our institution between January 1983 and December 1991. Grades 0 and 1 were designated if there was no opacification of the pulmonary arteries or veins, whereas grades 2 and 3 were assigned if there was such opacification. The median age at repair was 8 days (range 1 day to 13 months) and the median age at catheterization was 11.2 months (range 3.6 to 58.5). An intact ventricular septum was present in 84 (71%) of 119 patients. RESULTS: Significantly increased bronchial flow (grade 2 or 3) was present in 55 (46%) of 119 patients. Age at repair, age at catheterization and interval between repair and catheterization were not associated with significantly increased bronchial flow; however, an intact ventricular septum was weakly associated with increased flow (p = 0.04). Coil embolization was performed in five patients with complete occlusion of the vessels and no significant complications. CONCLUSIONS: Abnormally enlarged bronchial arteries are frequently identified at postoperative catheterization despite early repair and may explain continuous murmurs or persistent cardiomegaly in patients with otherwise normal noninvasive findings. When clinically indicated, catheter-directed therapy can be performed with good results.

Intraaortic spring coil loops: early and late results.

OBJECTIVES: Our aim was to determine the late fate of intraaortic spring coil loops after embolization of aortopulmonary vessels. BACKGROUND: In some aortopulmonary collateral vessels and patent ductus arteriosi, the narrowest segment is close to the aorta; coils used to close such vessels will "straddle" the lesion, allowing one or more coil loops to protrude into the aortic lumen. The consequences of this procedure are unknown. METHODS: We reviewed the cineangiograms of all patients who had at least one aortopulmonary collateral vessel or patent ductus arteriosus closure between January 1, 1988 and August 31, 1993. From this group, 53 patients had multiple-plane angiographic evidence of intraaortic coil loops. All subsequent cineangiograms were reviewed to determine coil position or movement and evidence of recanalization or endothelial coverage of the coil loop. We also reviewed each hospital record or communicated directly with referring physicians to identify any subsequent complications such as emboli or endocarditis. RESULTS: Of the 53 patients with intraaortic coil loops, 49 patients had closure of one or more aortopulmonary collateral vessels (59 vessels), and 4 had closure of a patient ductus arteriosus (4 vessels). Patient follow-up ranged from 1 day to 66 months (median 20 months); follow-up was not available in 6 patients. Five of the 53 patients (9.3%; 95% confidence limits [CL] 3.1% to 20.7%) died at operation or of end-stage heart failure. Patients with late angiography had no residual flow in 31 of 35 aortopulmonary collateral vessels (88.6%; 95% CL 73.3% to 96.8%), and 0.5 mm separated the coil and aortic contrast column in all 12 coils with adequate angiography, suggesting endothelial coverage of the intraaortic coil loop. No episodes of stroke, embolic events, endocarditis or coil migration were reported. CONCLUSIONS: Although coil occlusion of aortopulmonary collateral vessels or patent ductus arteriosi may produce intraaortic coil loops, endothelialization appears routine. No late complications associated with intraaortic coil loops were observed.

Midterm results of balloon dilation of congenital aortic stenosis: predictors of success.

OBJECTIVES: We evaluated patient and procedural characteristics that influence the midterm success of balloon dilation of congenital aortic stenosis. BACKGROUND: Balloon dilation is a new treatment for congenital aortic stenosis. Factors that influence midterm success are unknown. METHODS: We performed a retrospective review of 148 children >1 month old who underwent balloon dilation for aortic stenosis. RESULTS: Balloon dilation was successful in 87% of patients, with a procedural mortality rate of 0.7%. The average immediate peak to peak gradient reduction was 56.4 +/- 19.9% (mean +/- SD). Prior valvotomy was the only factor that significantly reduced the immediate gradient reduction after dilation (47.1 +/- 21.8% vs. 57.8 +/- 19.6%, p < 0.01). Survival after dilation was 95% at 8 years. Seventy-five percent of patients were free of repeat intervention 4 years after dilation, whereas 50% remained free of repeat intervention at 8 years. Asymmetrically thick valve leaflets (risk ratio [RR] 0.17, p < 0.01) and prior aortic valvotomy (RR 0.35, p = 0.02) decreased the risk of repeat intervention. Aortic regurgitation grade > or = 3 (RR 4.27, p = 0.04) and residual gradient after dilation (RR 1.63 for 10 mm Hg, p < 0.01) increased the risk. CONCLUSIONS: The 8-year survival rate after dilation was 95% with 50% of patients free of repeat intervention. Factors that increased the risk for repeat intervention included symmetrically thin or thick aortic valve leaflets, regurgitation grade > or = 3 after dilation and a high residual gradient after dilation. The incidence of repeat intervention after dilation was high owing to its palliative nature.

Tetralogy of Fallot with diminutive pulmonary arteries: preoperative pulmonary valve dilation and transcatheter rehabilitation of pulmonary arteries.

OBJECTIVES: This study sought to determine the results of a novel transcatheter management approach in tetralogy of Fallot with diminutive pulmonary arteries. BACKGROUND: Tetralogy of Fallot with diminutive pulmonary arteries and severe pulmonary stenosis is rare and resembles tetralogy of Fallot with pulmonary atresia: There is a high incidence of aortopulmonary collateral channels, arborization abnormalities, stenoses and need for multiple operations. Because a combined catheter-surgery approach facilitates repair in these patients, such an approach may benefit those with diminutive pulmonary arteries and pulmonary stenosis. METHODS: Clinical, catheterization and surgical data were studied retrospectively for 10 such patients undergoing preoperative pulmonary valve balloon dilation, among other transcatheter interventions, from January 1989 to January 1995. RESULTS: Initially, the Nakata index ranged from 20 to 98 mm2/m2 (mean 67 +/- 28 mm2/m2). The pulmonary valve was first balloon dilated (mean balloon/annulus 1.5 +/- 0.3), and the mean initial valve annulus Z score (-4.0 +/- 1) increased to -33 +/- 1.1 (p < 0.01) Other interventions included branch pulmonary artery balloon dilation (7 patients, 23 vessels) and coil embolization of aortopulmonary collateral channels (8 patients, 31 collateral channels). At preoperative follow-up catheterization, the mean pulmonary annulus Z score was -3.1 +/- 0.7, and the Nakata index increased to 143 +/- 84 mm2/m2 (p < 0.03). All patients underwent complete surgical repair successfully. At a mean follow-up period of 2.6 +/- 2 years, right ventricular pressure was < 70% systemic in all patients and < 50% systemic in seven. CONCLUSIONS: In patients with tetralogy of Fallot, severe pulmonary stenosis and diminutive pulmonary arteries, initial pulmonary valve balloon dilation increases the annulus Z score and anterograde pulmonary blood flow and facilities simultaneous coiling of aortopulmonary collateral channels and access for branch pulmonary artery dilation, all of which results in pulmonary artery growth, simplifying surgical management.

Generalized arteriopathy in Williams syndrome: an intravascular ultrasound study.

OBJECTIVES: We used intraluminal ultrasound imaging to provide additional information about the vascular pathology in Williams syndrome. BACKGROUND: The cardiovascular pathology of Williams syndrome consists of medial hypertrophy in both systemic and pulmonary arteries, which results in lumen narrowing. METHODS: Systemic and pulmonary arteries were examined in vivo using intravascular ultrasound imaging (5F, 30-MHz catheter) in three children with Williams syndrome. RESULTS: All arteries exhibited severe wall thickening with secondary lumen narrowing. Balloon dilation of a branch pulmonary artery in two children resulted in a significant localized increase in lumen caliber associated with a tear in the vessel wall. CONCLUSIONS: Intravascular ultrasound imaging in patients with Williams syndrome may permit better understanding of the pathophysiology of the syndrome and a more rational approach to therapeutic interventions.

Coil embolization to occlude aortopulmonary collateral vessels and shunts in patients with congenital heart disease.

Gianturco coils were used to embolize 77 vessels in 54 patients: 58 aortopulmonary collateral vessels, 14 Blalock-Taussig shunts, 3 arteries involved in pulmonary sequestrations and 2 venae cavae. Embolization resulted in total occlusion in 53 (69%), subtotal occlusion in 19 (25%) and partial occlusion in 3 (1 intentional). Two embolizations failed to reduce flow. Thus, 72 (95%) of 76 embolizations in which complete occlusion was the intended result resulted in total or subtotal occlusion. Analysis of the results demonstrates that completely occluded collateral vessels were longer and had a smaller diameter than did incompletely occluded vessels. Complications included six cases of inadvertent embolization to the pulmonary arteries (n = 5) or the aorta (n = 1); three were retrieved and three were left without symptoms. In addition, there was a case of severe hemolysis after intentional partial occlusion of a Blalock-Taussig shunt. The results demonstrate that coil embolization can be an effective procedure for managing a wide variety of aorto-pulmonary collateral vessels and shunts in children with congenital heart disease.

Transvascular balloon dilation for neonatal critical aortic stenosis: early and midterm results.

OBJECTIVES: We evaluated our immediate and midterm (mean 4.3 years) results of balloon dilation of critical valvular aortic stenosis in 33 neonates. BACKGROUND: Balloon dilation has been used as an alternative to surgical treatment. Reports to date consist of small series (largest 16 babies) with short-term follow-up (longest 4.8 years). METHODS: From 1985 to 1991, 33 neonates had dilation at a mean age of 13 days and a mean weight of 3.4 kg. Nineteen of the neonates (58%) were intubated and received prostaglandins, and 94% had other cardiac abnormalities. RESULTS: The dilation was completed retrograde in 31 of the neonates (umbilical artery in 11 and femoral artery in 20) and anterograde (femoral vein) in 2. The average immediate peak gradient and left ventricular end-diastolic pressure reductions were 54% and 20%, respectively. The overall mortality rate was 12% (three early deaths and one late). All 20 neonates dilated through a femoral artery initially had pulse loss with restoration in 35% after thrombolytic therapy. At 8.3 years, survival and freedom of reintervention probability rates were 88% and 64%, respectively. At mean 4.3 years of follow-up, 83% of the survivors were asymptomatic; Doppler study revealed a maximal instantaneous gradient < 50 mm Hg in 65% of neonates and significant aortic regurgitation in 14%. CONCLUSIONS: This study confirms that dilation of aortic stenosis in neonates is effective; reintervention (mostly redilation) is frequent (40%); and midterm survival is encouraging (88%).

Transcatheter umbrella closure of valvular and paravalvular leaks.

OBJECTIVES: Our aim was to adapt the technique of transcatheter umbrella closure of intracardiac defects for closure of valvular and paravalvular defects. BACKGROUND: The double-umbrella device developed by Rashkind and Cuaso has been safely and effectively delivered across a host of intracardiac defects, but transcatheter closure of valvular and paravalvular leaks has not been reported. METHODS: Between February 1987 and September 1990, eight patients who were believed to be poor operative candidates were taken to the catheterization laboratory for transcatheter double-umbrella closure of a valvular or a paravalvular leak. Four patients had a paravalvular leak around a prosthetic aortic valve. The other four patients had a valvular leak: one patient with a regurgitant native aortic valve after a Stansel procedure and three patients with a regurgitant porcine valve in a left ventricular apex to descending aorta conduit. RESULTS: Placement of a double-umbrella device was attempted in seven of the eight patients and was successful in all seven. Device placement was not attempted in one patient because of the crescentic shape of his defect. Two patients required two devices for each closure; the other five required only one device each. Angiography, performed on six patients after device closure, demonstrated that three patients had a completely occluded defect, two had trivial residual flow and one patient had mild residual flow through the device. All significant complications occurred in one patient who had hemolysis and oliguria that resolved when the initial umbrella was replaced by a larger device. In addition, two devices migrated to the patient's pulmonary arteries but were retrieved in the catheterization laboratory without difficulty. No other early or late complications occurred in 21 to 50 months of follow-up. Of the four patients with a paravalvular leak, the one who did not receive a device died at operation, one patient died at operation for an associated defect (in the operating room the umbrella was found securely in place across the paraaortic defect) and two patients are clinically well at home after 21 and 32 months, respectively. Of the four patients with closure of a valvular leak, one patient remains well at home 50 months later, one patient died at operation for associated defects and two patients had additional successful surgical treatment and remain well 29 months after device placement. CONCLUSIONS: Transcatheter umbrella closure appears to be a reasonable alternative for closure of a valvular or paravalvular leak in patients who are poor operative candidates.

Front-loading of double-umbrella devices, a new technique for umbrella delivery for closing cardiovascular defects.

In all, 22 defects (6 atrial septal, 13 ventricular septal, 2 patent ductus arteriosus and 1 coronary artery fistula) in 17 patients were closed with double umbrella using a new delivery technique, front-loading. The umbrella was loaded in the sheath outside the body and then advanced with-out use of the rigid pod to the defect. The defect was successfully closed in all 17 patients, including 5 in whom standard delivery techniques were unsuccessful. Front-loading enables the double umbrella to be delivered through a smaller long sheath than does the standard technique, and enables the flexible umbrella rather than the rigid pod to be advanced through curves in the sheath, and may improve delivery of double-umbrella devices to inaccessible parts of the circulation.

Interventional catheterization in pediatric congenital and acquired heart disease.

The present status of catheter-directed therapy in pediatric congenital and acquired heart disease is reviewed. Balloon pulmonary valvotomy, established as the procedure of choice for children and adults with isolated pulmonary stenosis, has now been extended to neonates with critical pulmonary stenosis. Balloon mitral and aortic valvotomy are being performed for congenital and acquired stenoses, although indications and optimal techniques remain to be established. Balloon angioplasty of coarctation effectively relieves obstruction in native and post-operative aortic arch stenoses, but remains controversial due to late aneurysm formation. Balloon angioplasty of branch pulmonary artery hypoplasia and stenosis, despite a relatively low success rate and significant complications, remains the procedure of choice due to lack of better therapeutic options. Transcatheter closure of congenital and acquired vessels and defects is being performed with increasing frequency. Embolization of aortopulmonary collaterals and shunts using Gianturco coils is safe and effective in selected patients. Although Rash-kind umbrellas are most commonly used for closure of a patent ductus arteriosus, their use, especially in closing intracardiac defects, remains investigational.

Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum.

Coronary artery pathology is a major determinant of treatment strategy and outcome in patients with pulmonary atresia and intact ventricular septum (PA/IVS). For this reason, infants with PA/IVS routinely undergo preoperative cardiac catheterization. The goal of this study was to identify echocardiographic predictors of coronary artery pathology in infants with PA/IVS. The initial preoperative echocardiograms of 30 consecutive infants with PA/IVS (median age at diagnosis 1 day) were reviewed for indexes predicting the degree of coronary pathology. The tricuspid valve (TV) annulus diameter Z- score was determined and evidence of abnormal flow in the coronary arteries by Doppler was evaluated. Coronary pathology was defined by angiography and graded as: 0 = no fistulae; 1 = fistulae/no right ventricular (RV)-dependent coronary arteries; 2 = fistulae with 1 RV-dependent coronary; 3 = fistulae with >/=2- vessel RV-dependent coronary arteries. Outcome was classified as: 2 ventricles, "1.5" ventricles, and 1 ventricle. By angiography, 30% of the patients had grade 0 coronary pathology, 30% had grade 1, 20% had grade 2, and 20% had grade 3. There was 1 death in a patient with grade 3 coronary pathology. Among the survivors (median age at follow-up 28. 6 months), biventricular circulation existed in 12 patients (41%), 7 patients (24%) were 1.5, and 10 (34%) were 1 ventricle. All patients with TV Z-score -2.5. The sensitivity, specificity, positive, and negative predictive values of TV Z-score

Coronary artery abnormalities detected at cardiac catheterization following the arterial switch operation for transposition of the great arteries.

Because the arterial switch operation has become the routine surgical approach for transposition of the great arteries, there is increasing awareness of adverse sequelae in some survivors. For the arterial switch to be considered the procedure of choice for transposition of the great arteries, long-term patency and normal function of the translocated coronary arteries must be achieved. We reviewed the cineangiograms and hemodynamic data in 366 patients who underwent postoperative catheterization after arterial switch operation. Of these, 13 patients (3%) had previously unsuspected coronary abnormalities diagnosed angiographically. No patient had noninvasive evidence of resting systolic dysfunction. Findings included left main coronary artery stenosis (n = 3) or occlusion (n = 2), anterior descending coronary artery stenosis (n = 1) or occlusion (n = 2), right coronary artery stenosis (n = 1) or occlusion (n = 1), and small coronary artery fistulas (n = 3). One patient died suddenly 3.3 years after surgery, 1 patient is lost to follow-up, and the remaining 10 patients are alive and asymptomatic up to 11 years after surgery.

Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect.

A severely restrictive atrial septal defect (ASD) in neonates with hypoplastic left heart syndrome (HLHS) results in pulmonary venous hypertension, pulmonary edema, and intractable hypoxia. Between January 1983 and June 1998, 21 of 355 neonates presenting with HLHS (5.9%) underwent cardiac catheterization at median age 1 day (range 0 to 25), for creation or enlargement of a restrictive or absent interatrial communication. One patient died during preliminary angiography. Three underwent blade septostomy with 2 procedure-related deaths, and 1 had balloon atrial septostomy (BAS); all 4 died before surgical intervention. Fifteen underwent Brockenbrough atrial septoplasty with transatrial needle puncture and serial balloon dilations of the new ASD, 5 after unsuccessful BAS. The most recent patient had a stent placed across the atrial septum after transatrial needle puncture. In the 16 patients treated with septoplasty or stent, oxygen saturation increased from 50 +/- 4% to 83 +/- 2% (p <0.0001) and transatrial pressure gradient decreased from 16 +/- 1 to 6 +/- 1 mm Hg (p <0.0001). One patient died awaiting transplantation, supportive care only was requested in 1, and 14 underwent stage 1 palliation. Eight of 14 (57%) survived to hospital discharge. Six of 7 (86%) survived bidirectional Glenn and the 3 who have undergone fenestrated Fontan are alive. In neonates with HLHS, a restrictive ASD resulting in profound cyanosis demands urgent intervention. BAS is frequently unsuccessful and blade septostomy has high mortality. Pulmonary venous hypertension can be adequately relieved by Brockenbrough atrial septoplasty or stenting, allowing stabilization before reconstructive surgery or while awaiting transplant.

Balloon aortic valvuloplasty in the young adult with congenital aortic stenosis.

Although balloon aortic valvuloplasty (BAV) is effective in the acute management of valvar aortic stenosis (AS), sustained benefit of this technique has been shown to vary in differing age groups. The role of BAV in the young adult with congenital, nondegenerative AS is poorly defined. The catheterization results and follow-up echocardiographic data were reviewed for all patients (n = 18) between the ages of 17 and 40 years (mean 23 +/- 7) undergoing BAV at our institution between March 1986 and January 1992. Peak-to-peak systolic ejection gradient was reduced by 55%, from 85 +/- 29 to 38 +/- 17 mm Hg (p < 0.001). Aortic valve area increased from 0.9 +/- 0.2 to 1.1 +/- 0.3 cm2 (p = 0.003). Results of dilation were inadequate in 2 patients. There were no deaths, myocardial infarction, or embolic events. The 16 "effectively" dilated patients were followed with serial echocardiography for a period of 1 to 82 months, demonstrating persistent gradient relief in most patients (maximal instantaneous gradient at follow-up, 55 +/- 17 vs 79 +/- 22 mm Hg before dilation, p < 0.001). Aortic valve replacements were performed in 5 patients, 2 with unsuccessful initial dilations. At the time of the most recent echocardiogram, 8 of 16 patients remained "incident free," with no subsequent catheterization or surgical interventions, a maximal instantaneous gradient of < or = 55 mm Hg, no more than moderate aortic regurgitation, and preserved ventricular function.(ABSTRACT TRUNCATED AT 250 WORDS)