Jay M. Levy

Role of buttoned double-disc device in the management of atrial septal defects

Abstract Sixteen patients seen over a 9-month period ending in August 1990 were offered transcatheter closure of their ASD with a custom-made “buttoned” double-disc device. The study was approved by the Institutional Review Board and informed consent was obtained in each case. The device consists of an occluder, a counteroccluder, and a loading wire and is delivered to the ASD site via an 8F sheath. Parents of two children elected surgical closure. In five children the stretched diameter of the ASD was too large (>20 mm) and transcatheter closure was not attempted. These seven children underwent elective surgical closure without incident. In one child the defect measured 5 mm and the Qp:Qs was 1.4:1 and therefore ASD closure was not recommended. In the remaining eight children transcatheter closure was attempted. In two of the children the occluder pulled through the ASD and was successfully retrieved and the children later underwent uneventful elective surgical closure. The device was implanted across the ASD in six children. In one child the device dislodged from the ASD site within minutes after implantation and the child was sent to emergency surgery, where the device was removed and the ASD was closed. In the remaining five patients, aged 7 months to 45 years (weight 3.6 to 50 kg), with a Qp:Qs range of 1.3 to 2.3 and a stretched diameter of 10 to 19 mm, the ASD closure was successful with 25 to 40 mm size devices. Repeat echo-Doppler studies 2 weeks and 3 months after the procedure in all patients and 6 months later in two children did not reveal any residual shunt. It is concluded that (1) the custom-made “buttoned” double-disc device can be implanted across the ASD safely and effectively via an 8F sheath, thus making transcatheter ASD closure feasible even in very young infants; (2) measurement of stretched diameter of the ASD in the catheterization laboratory is a useful guide for selection of an appropriate-sized device; and (3) additional clinical trials are warranted to confirm the efficacy and safety of the device.

Intermediate-term follow-up results of balloon aortic valvuloplasty in infants and children with special reference to causes of restenosis

Sixteen infants and children with valvular aortic stenosis underwent percutaneous balloon aortic valvuloplasty over a 36-month period ending August 1988. The mean systolic pressure gradient across the aortic valve decreased from 72 +/- 21 (mean +/- standard deviation) to 28 +/- 13 mm Hg (p less than 0.001) immediately after valvuloplasty; the degree of aortic insufficiency did not significantly increase. Follow-up catheterization (in 10 patients) and Doppler data (in all 16 patients) were available 3 to 32 months (mean 12 months) after valvuloplasty and revealed a residual aortic valvular gradient of 37 +/- 23 mm Hg, which continues to be significantly lower (p less than 0.001) than that before valvuloplasty. There was no increase in aortic insufficiency. On the basis of follow-up data, the 16 children were divided into 2 groups: group I with good results (gradients less than or equal to 49 mm Hg), 12 patients; and group II with poor results (gradients greater than or equal to 50 mm Hg), 4 patients. All 4 patients in group II required repeat balloon valvuloplasty or surgical valvotomy; none from group I required these procedures. Seventeen general, anatomic, physiologic and technical variables were examined by a multivariate logistic regression analysis to identify factors associated with restenosis; these risk factors were: age less than or equal to 3 years; and immediate aortic valvular gradient after valvuloplasty greater than or equal to 30 mm Hg. The immediate and intermediate-term follow-up results of balloon aortic valvuloplasty are encouraging. Recognition of the risk factors may help identify potential candidates for recurrence.(ABSTRACT TRUNCATED AT 250 WORDS)

Balloon angioplasty of stenosed Blalock-Taussig anastomosis: Role of balloon-on-a-wire in dilating occluded shunts

Six children with cyanotic congenital heart defects, aged 6 to 60 months, underwent percutaneous balloon angioplasty of a narrowed Blalock-Taussig (BT) shunt to improve arterial oxygen saturation. The indication for angioplasty was a cyanotic heart defect not amenable to total surgical correction, either because of the age and size at presentation or because of anatomic complexity, but at the same time requiring palliation of pulmonary oligemia. Following balloon angioplasty, there was an increase in arterial oxygen saturation from 71 +/- 8% to 81 +/- 6% (p less than 0.05). In one child with long segment narrowing, there was no significant improvement in oxygen saturation, and this child underwent an additional BT shunt on the contralateral side. On follow-up 3 to 12 months after balloon angioplasty, the oxygen saturations remained improved (78 +/- 10%) in the remaining five patients. In two children with either complete or almost complete blockage of the BT shunt, we were unable to advance any catheter across the shunt but we were able to advance a 2 or 3 mm balloon on a wire and dilate the shunt, followed by introduction of a catheter carrying a larger balloon for angioplasty. This has permitted us to obtain the pulmonary artery pressure directly (this information was of obvious value in patient management) and resulted in an improvement in the arterial oxygen saturation. It is concluded that (1) balloon angioplasty of narrowed BT shunts is feasible, effective, and safe and (2) even completely occluded shunts can be cannulated and the balloon dilated with the newly available balloon-on-wire devices.

Tricuspid atresia: Association with persistent truncus arteriosus

Clinical, echo-Doppler, and pathologic data of a rare case of tricuspid atresia in association with truncus arteriosus are presented. There are only six patients (including the patient reported here) with this anomaly in whom detailed pathologic and/or clinical descriptions are available in the literature. An additional six cases were mentioned in the literature but without any details. A prevalence rate of 1.4% among tricuspid atresia cases is estimated. All infants presented with symptoms of cyanosis and/or congestive heart failure within a few days to 2 months after birth and died shortly thereafter. Cardiomegaly and increased pulmonary vascular markings on the chest x-ray film and left axis deviation with left ventricular hypertrophy on the ECG are usually present. Echocardiographic, catheterization, and angiocardiographic studies are helpful in documenting anatomic and physiologic features of this anomaly. Pathologically, the muscular type of tricuspid atresia, type I or II truncus arteriosus, a large subtruncal VSD, and a hypoplastic right ventricle are present. It is concluded that coexistence of tricuspid atresia and truncus arteriosus is rare; clinical, laboratory, and pathologic features are distinctive; the clinical presentation early in life with rapid demise is common; and prompt palliative surgery immediately after recognition of this anomaly should be undertaken.

The use of the diving reflex to terminate supraventricular tachycardia in a 2-week-old infant.

The use of the diving reflex to terminate a case of paroxysmal supraventricular tachycardia (PST) is described in a 2-week-old infant who presented in severe congestive heart failure with supraventricular tachycardia at a rate of 300. The infants face was placed in a basin of ice water at 5 degrees C. for 5 seconds with manual occlusion of the infants nostrils to prevent aspiration. The PST converted to a sinus rhythm of 120 within 3 seconds of facial immersion. The physiology of the diving reflex is reviewed and the uses and hazards of this reflex in terminating attacks of PST in infants is discussed.

Takayasu's arteriopathy with involvement of aortic valve and bacterial endocarditis

Summary A case of giant cell arteritis complicated by cardiac valvular involvement and myocarditis with superimposed bacterial endocarditis and rupture of a valve cusp in a 13-year-old child is reported. This sequence of events has not been previously recorded. The clinical manifestations closely simulated rheumatic heart disease, although pulselessness was a prominent feature. Recognition of this disease, although rare in the pediatric age group, is important since corticosteroid therapy may afford improvement.

99mTechnetium pyridoxylidene glutamate imaging in visceral heterotaxy (Ivemark's syndrome).

A two and one-half year old boy with complex congenital heart disease had Howell-Jolly bodies in his blood raising the possibility of the asplenia syndrome. A 99mTc-sulfur colloid liver-spleen scan was reported normal, but the right lobe of the abnormally reversed liver was mistaken for the spleen. The diagnosis of visceral heterotaxy (Ivemarks syndrome) was established after scanning the patient with a new radiopharmaceutical, 99mTechnetium pyridoxylidene glutamate. The agent clearly demonstrated a left-sided gall bladder, and on comparison with the sulfur colloid scan it was established that asplenia was present. Radionuclide imaging with the new generation of hepatobiliary agents is a reliable method to document asplenia and is useful in studying patients with visceral heterotaxy.

A Better Surgical Technique for Repairing Pulmonary Arteries after Banding

A technique is presented to eliminate the residual gradient more completely after removing the main pulmonary band at the time of primary repair of ventricular septal defect. The band and underlying pulmonary artery are circumferentially excised, except for a small posterior part, and pulmonary artery is reanastomosed.

Intrapulmonary Steal Complicating Repair of Tetralogy of Fallot

A patient with total occlusion of the distal right pulmonary artery secondary to the performance of a Waterston shunt is reported. A nearly fatal intrapulmonary steal occurred at the time of corrective operation when an attempt was made to revascularize both pulmonary arteries.