Balloon dilation angioplasty of peripheral pulmonary stenosis associated with Williams syndrome.

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.

Left superior vena cava connection to unroofed coronary sinus associated with positional cyanosis: successful transcatheter treatment using Gianturco-Grifka vascular occlusion device.

A persistent left superior vena cava connection to an unroofed coronary sinus is a rare cardiac anomaly that is associated with a variable degree of cyanosis. We report an infant with this condition and the unusual feature of cyanosis dependent on head position. When the patient's head was rotated to the left, he developed severe stenosis of the left internal jugular vein, enlarged cervical collateral veins that connected to the right superior vena cava and had an oxygen saturation 95%. When the patient's head was rotated to the right, the left internal jugular vein was widely patent and systemic oxygen saturation decreased to 87%. There was no right ventricular volume overload. Temporary occlusion of the left superior vena cava documented tolerable proximal venous pressure. Cyanosis was relieved by transcatheter closure of the left superior vena cava with a Gianturco-Grifka vascular occlusion device. Cathet. Cardiovasc. Intervent. 48:369-373, 1999.

Ebstein's anomaly associated with severe valvar pulmonary stenosis: successful palliation with balloon pulmonary valvuloplasty in an adult.

We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention.

Repair of coarctation of the aorta during infancy minimizes the risk of late hypertension.

BACKGROUND: Recent surgical reports on coarctation of the aorta have primarily focused on the relative merits of various operative techniques. However, appropriate timing for elective repair remains unclear. METHODS: In a retrospective analysis we examined the surgical outcomes in 176 consecutive patients undergoing repair of coarctation of the aorta in our institution over a 25-year period. Ninety-nine percent of the patients had follow-up for a median of 7.5 years. RESULTS: A total of 13 patients have died (7.4% overall mortality). Nine of these patients had associated complex intracardiac anomalies. There was no mortality in the 113 patients with isolated coarctation. Residual or recurrent coarctation occurred in 27 patients (15.3%). The age at operation and the type of surgical repair did not have an effect on the incidence of recurrence. Persistent or late hypertension was identified in 18 of the 107 patients who have been followed up for more than 5 years (16.8%). A total of 48 patients operated on during infancy have been followed up for more than 5 years. Only 2 have developed late hypertension (4.2%). Both of these patients had recurrence. In contrast, 16 of the 59 patients operated on after a year of age had late hypertension (27.1%). CONCLUSIONS: To minimize the risk of persistent hypertension, elective repair of coarctation should be performed within the first year of life.

Effect of pulmonary artery stenoses on the cardiopulmonary response to exercise following repair of tetralogy of Fallot.

Data from exercise tests, echocardiograms, and lung perfusion scans were analyzed to determine whether the excessive minute ventilation (VE) often encountered among patients with tetralogy of Fallot is due to ventilation-perfusion mismatch secondary to branch pulmonary artery stenoses. Patients with branch PA stenoses had lower peak oxygen consumptions and higher VE during exercise than did patients without stenoses, and a strong correlation existed between the degree of pulmonary blood flow maldistribution on lung perfusion scan and the amount of excessive VE during exercise.

Reduced incidence of ventricular ectopy with a 4F Halo catheter during pediatric cardiac catheterization.

Catheter-induced ventricular ectopy can limit assessment of contractility or anatomy. We compared the incidence of ventricular ectopy in infants and children undergoing left ventriculography using a 4F Halo catheter or a 4F pigtail catheter. For each group, 17 patients had 19 ventriculograms. Iopamidol 76% was used for each study. There was no statistical difference between the Halo and pigtail groups (mean+/-SEM) for age (16.2+/-3.8 vs. 12.9+/-1.8 mo), weight(8.1+/-1.0 vs. 8.0+/-0.7 kg), gender, left ventricular end diastolic pressure (9.6+/-1.1 vs. 9.8+/-1.3 mm Hg), catheter location in the ventricle, or volume of contrast (1.31+/-0.06 vs. 1.35+/-0.06 cc/kg). The Halo group had a more rapid injection rate (1.42+/-0.15 vs. 0.99+/-0.10 cc/kg/sec, P< 0.02). The Halo group had a lower overall incidence of ventricular ectopy (3 vs. 13 studies, P < 0.001), ventricular ectopy >1 beat (1 vs. 10 studies, P < 0.002), and ventricular couplets or tachycardia (1 vs. 9 studies, P < 0.008). The 4F Halo catheter is associated with less ventricular ectopy than a 4F pigtail catheter and should be considered for use during pediatric catheterization.

Excessive anaerobic metabolism during exercise after repair of aortic coarctation.

OBJECTIVES: To determine whether survivors of surgery for coarctation of the aorta (CoA) have an excessive reliance on anaerobic metabolism during exercise. BACKGROUND: Patients with peripheral vascular disease cannot increase blood flow to their muscles normally during exercise. Consequently they acquire an early, excessive reliance on anaerobic metabolism and have depression of the ventilatory anaerobic threshold (VAT) and of the slope of the oxygen consumption-work rate relationship (delta VO2/delta WR). We speculated that the capacity to augment blood flow to the lower extremities during exercise may be impaired after CoA surgery and would result in similar metabolic disturbances. STUDY DESIGN: Progressive exercise tests were performed on 15 patients (ages 19 +/- 7 years; range, 10 to 32) after successful repair of CoA (residual resting gradient, 7.7 +/- 7.1 mm Hg; range, 0 to 18), 15 age- and sex-matched healthy control subjects, and 10 patients (ages 13 +/- 3 years; range, 10 to 20) who had undergone ligation of a patent ductus arteriosus. RESULTS: The CoA patients' VAT averaged 14.8 +/- 3.8 ml O2/kg per minute versus 19.3 +/- 3.1 ml O2/kg per minute for the control subjects (p < 0.01), and their delta VO2/delta WR averaged 8.2 +/- 1.8 ml/watt compared with 10.1 +/- 1.4 ml/watt for control subjects (p < 0.01). Furthermore, 10 of 15 CoA patients had a VAT of less than 40% of predicted maximal oxygen consumption, and 9 of 16 had a delta VO2/delta WR of less than 8.7 ml O2/watt (generally accepted abnormal values). Patients with patent ductus arteriosus resembled the healthy control subjects with regard to anaerobic metabolism during exercise. CONCLUSIONS: Patients who have had CoA repairs commonly manifest an excessive reliance on anaerobic metabolism during exercise. This phenomenon may result from persistent blood flow abnormalities across the aortic arch during exercise, which may be present even after apparently successful surgery.

Effect of multiple coil closure of patent ductus arteriosus on blood flow to the left lung as determined by lung perfusion scans.

Lung perfusion scans reveal that multiple-coil closure of the ductus does not interfere with pulmonary blood flow. However, caution is necessary when placing multiple coils in small infants.

Transcatheter closure of patent ductus arteriosus using coils.

Ninety-seven of 100 patients had a successful attempt at coil closure of the patent ductus arteriosus. For all 46 patients in whom a single coil and in 47 patients in whom multiple coils were placed, there was immediate complete closure that persisted at a median follow-up period of 1.9 years.

Balloon atrial septostomy using a new low-profile balloon catheter: initial clinical results.

Balloon atrial septostomy remains an important interventional procedure in the pediatric age group. The incidence of potential complications using the conventional balloon ranges from 0-11%. The purpose of this study was to evaluate a new low-profile end-hole septostomy balloon catheter with dual lumen, inserted via a 5F or 6F sheath. Seventeen neonates and infants with various forms of congenital heart disease requiring palliation underwent septostomy using the new catheter at a median age of 19 days (with a range of 1-593 days), and a median weight of 3.4 kg (a range of 2.5-8.4 kg). The aortic saturation increased from 72 +/- 20% to 87 +/- 7%, p < .001; and the gradient across the atrial septum decreased (a-wave gradient from 11.2 +/- 10.3 to 2.1 +/- 3.6 mm Hg; v-wave from 10.4 +/- 7.7 to 1.2 +/- 1.2 mm Hg; and mean gradient from 8.5 +/- 6.9 to 0.9 +/- 1.3 mm Hg, p < .002). The diameter of the defect increased from 2.7 +/- 1.7 mm to 8 +/- 2.3 mm, p < .001. There were no complications. We conclude that this new low-profile septostomy catheter is safe and effective in creating a large defect size between the atria. Because of the smaller inflation size of the balloon and smaller introducer sheath compared with the conventional catheter, this new septostomy catheter should be especially useful in small neonates.

Update on the modified Fontan procedure.

There have been multiple modifications for surgical palliation of functional single ventricle since the initial report by Fontan and Baudet in 1971. A modified Fontan procedure has been offered to patients with a variety of complex cyanotic heart disease at younger ages, and has resulted in decreased mortality. Reducing morbidity continues to be a challenge. These issues are multiple and include effusions, arrhythmia, ventricular function, exercise ability, progressive cyanosis from pulmonary arteriovenous malformations, systemic to hepatic venovenous malformations or atrial level shunting, thromboembolism, and protein-losing enteropathy. This article reviews clinical contributions published in the past year.

Electrocardiographic markers of late sudden death risk in postoperative tetralogy of Fallot children.

Following surgery for tetralogy of Fallot (TOF), children may develop late onset ventricular arrhythmias. Many patients have both depolarization and repolarization abnormalities, including right bundle branch block (RBBB) and QT prolongation. The goal of this study was to improve prospective risk-assessment screening for late onset sudden death. Resting ECG markers including QRS duration, QTc, JTc, and interlead QT and JT dispersion were statistically analyzed to identify those patients at risk for ventricular arrhythmias and sudden cardiac death. To determine predictive markers for future development of arrhythmia, we examined 101 resting ECGs in patients (age 12 +/- 6 years) with postoperative TOF and RBBB, 14 of whom developed late ventricular tachycardia (VT) or sudden death. These ECGs were also compared with an additional control group of 1000 age- and gender-matched normal ECGs. The mean QRS (+/- SD) in the VT group was 0.18 +/- 0.02 seconds versus 0.14 +/- 0.02 seconds in the non-VT group (P < 0.01). QTc and JTc in the VT group was 0.53 +/- 0.05 seconds and 0.33 +/- 0.03 seconds compared with 0.50 +/- 0.03 seconds and 0.32 +/- 0.03 seconds in the non-VT group (P = NS). There was no increase in QT dispersion among TOF patients with VT or sudden death compared with control patients or TOF patients without VT, although JT dispersion was more common in the TOF groups. A prolonged QRS duration in postoperative TOF with RBBB is more predictive than QTc, JTc, or dispersion indexes for identifying vulnerability to ventricular arrhythmias in this population, while retaining high specificity. The combination of both QRS prolongation and increased JT dispersion had very good positive and negative predictive values. These results suggest that arrhythmogenesis in children following TOF surgery might involve depolarization in addition to repolarization abnormalities. Prospective identification of high-risk children may be accomplished using these ECG criteria.

Possible new autosomal recessive syndrome of lymphedema, hydroceles, atrial septal defect, and characteristic facial changes.

We describe two brothers with congenital lymphedema of lower limbs, atrial septal defect (ASD), and similar facial appearance. A sister had severe hydrops fetalis, ASD, omphalocele, and other anomalies. This combination of congenital lymphedema and ASD differs from other reported cases of congenital lymphedema and most likely constitutes a previously unrecognized autosomal recessive syndrome.

Transcatheter closure of large patent ductus arteriosus (> or = 4 mm) with multiple Gianturco coils: immediate and mid-term results.

OBJECTIVE: To assess the immediate and mid-term results of transcatheter closure of patent ductus arteriosus (PDA) > or = 4 mm with multiple Gianturco coils. (Transcatheter closure of large PDAs using the Rashkind occluder or the buttoned device is associated with a 7-38% incidence of residual shunt.) METHODS: 19 patients (7 male, 12 female) underwent an attempt at anterograde transcatheter closure with multiple Gianturco coils of a large PDA at a median age of 3.8 yr (range 2 weeks-34 yr) and median weight of 14 kg (range 2.3-80 kg). RESULTS: The median PDA diameter at the narrowest segment was 4.3 mm (range 4-7 mm) and the mean (SD) Qp/Qs was 1.9 (0.8). Each patient had left atrial and left ventricular volume overload. A 4F catheter was used to deliver the coils in all patients. There was immediate and complete closure in 16/18; one patient had residual shunt that was closed at a second procedure and the other had spontaneous disappearance of the residual shunt at the six week visit. A short ductus (angiographic type B) in one patient could not be closed. The median number of coils placed at the first attempt to close the ductus was 4 (range 2-6 coils) and the median fluoroscopy time was 40 minutes (range 13-152 minutes). Mild left pulmonary artery stenosis occurred in the two smallest patients. Coil migration to the lung occurred in 3 patients with retrieval of coils in two patients. All procedures but one were done on an outpatient basis. At a median follow up of 1.6 yr (range 2 weeks-2.2 yr) all patients had complete closure with no new complications. CONCLUSIONS: Anterograde transcatheter closure with multiple Gianturco coils is an effective treatment for most patients with large PDA of diameters up to 7 mm. This technique can be performed in small infants on an outpatient basis without the need for general endotracheal anaesthesia.

Transcatheter closure with single or multiple Gianturco coils of patent ductus arteriosus in infants weighing < or = 8 kg: retrograde versus antegrade approach.

Experience with transcatheter closure (TCC) of patent ductus arteriosus (PDA) in small infants is limited. Our goals were to evaluate the immediate and short-term results of TCC of PDA in small infants (< or = 8 kg) as attempted with single or multiple Gianturco coils and to compare results for PDA < or = 2.5 mm versus PDA > 2.5 mm. Twenty-four infants underwent an attempt at TCC of PDA at a median age of 0.7 years (range 2 weeks to 1.5 years) and median weight of 6.5 kg (range 2.3 to 8 kg). Coils were placed by the transvenous route in 14 patients and by the transarterial route in 10 patients. The median PDA diameter was 2.7 mm (range 0.4 to 5.2 mm). Ten patients had PDA diameters < 2.5 mm and all had complete closure, 9 with a single coil and 1 with two coils. Fourteen patients had PDA diameters > or = 2.5 mm; closure was complete in 10 patients, 9 of whom required multiple coils. There was a trend toward improved results with multiple coils delivered transvenously. There was immediate complete closure of the PDA with a diameter as large as 5.2 mm that persisted at a median follow-up period of 1.2 year. Closure was unsuccessful in 4 patients, each of whom required multiple coils. The median fluoroscopy time was 19 minutes. Complications included transient loss of femoral pulse in 2 infants, mild left pulmonary artery obstruction in 2, and nonretrievable coil migration to the right lung in one. We conclude that TCC is effective therapy for small infants with a PDA of diameter < or = 5.2 mm by the single-or multiple-coil technique on an outpatient basis and that TCC can be performed in neonates as small as 2.3 kg.

Stent implantation for relief of pulmonary artery stenosis: immediate and short-term results.

Our objective was to assess the immediate and short-term results of stent implantation to relieve pulmonary artery stenosis (PAS). Thirty-seven patients underwent an attempt at stent implantation at a median age of 7.0 years (range, 0.8-31.4 years) and a median weight of 20.5 kg (range, 7.4-85 kg). Twenty-two patients had previous tetralogy of Fallot repair. A total of 55 stents were implanted successfully in 36 patients. The peak systolic gradient across the stenotic segment decreased from a mean of 43 +/- 20.4 mmHg prestent to 13 +/- 13.9 mmHg (P < 0.001) poststent. The diameter of the narrowest segment increased from a mean of 4.8 +/- 1.6 mm to 10.5 +/- 2.6 mm (P < 0.001). The right ventricular-to-aortic mean systolic pressure ratio decreased from 0.74 +/- 0.2 to 0.52 +/- 0.19 (P < 0.001). Complications included balloon rupture prior to full stent expansion in 4 patients (in 2 patients the stent was positioned in the superior vena cava, and in 2 in the inferior vena cava), distal migration of a stent which was successfully retrieved at surgery 1 mo later in 1 patient, and tethering of the stent to the balloon requiring surgical removal in 1 patient. One patient died several hours after stent placement. Sixteen patients underwent repeat catheterization at a mean follow-up interval of 0.9 +/- 0.5 years (range, 0.2-2.0 years). The mean gradient across the stent for these 16 patients was 26.7 +/- 19.8 mmHg, and there was no change in the mean diameter (9.4 +/- 3.2 mm). Two patients developed stenosis related to neointimal proliferation at the stent site which was redilated successfully. In conclusion, stent implantation is generally safe and effective in relieving PAS.

Interventional cardiac catheterization therapy for combined coarctation of the aorta and patent ductus arteriosus: successful outcome in two infants.

We report two infants each with coarctation of the aorta and small patent ductus arteriosus who had balloon angioplasty therapy for aortic obstruction at 3-6 months of age followed by anterograde placement of a single Gianturco coil to close the ductus at a separate catheterization. One infant developed recoarctation that resolved with repeat angioplasty prior to closure of the ductus. Follow-up evaluations have demonstrated a trivial aortic gradient, no aortic aneurysm, and no residual shunt.