Outpatient closure of the patent ductus arteriosus.

We have modified and applied to selected outpatients the transvenous approach to correction of patent ductus arteriosus (PDA) with the Rashkind PDA Occluder. Modifications included establishing the diagnosis and PDA anatomy before catheterization with echocardiography. The sedation/anesthetic regimen was altered to meet the needs with respect to transcatheter PDA closure rather than diagnostic cardiac catheterization.

Late results in patients with tetralogy of Fallot repaired during infancy.

Between 1973 and 1985, 220 infants (age 1 day to 18 months, mean = 7 months) underwent repair of tetralogy of Fallot (TOF) or TOF/pulmonary atresia with 17 early deaths. Follow-up status was ascertained for 184 of 203 survivors (91%) at a postoperative interval of 2 to 185 months (mean = 60 months). Significant residual hemodynamic lesions included ventricular septal defect in three, and right ventricular outflow gradient in excess of 40 mm Hg in 24.

Acute paralysis from inhaled barium carbonate.

A young chrome-plating worker suffered life-threatening hypokalemic paralysis when barium powder, used in cleaning the chrome tanks, blew back into his face.

Balloon dilation of critical pulmonary stenosis in the first week of life.

Although balloon dilation of valvular pulmonary stenosis is established in infants and children, the techniques for and results of balloon dilation in neonates with critical pulmonary stenosis remain largely unreported. Since January 1, 1985, six successive neonates with critical pulmonary stenosis (aged 1 to 6 days) underwent attempted balloon dilation. Each was cyanotic and three of the six were on prostaglandin E1 therapy and three required tracheal intubation and ventilation.

Lid-opening apraxia in Wilson's disease.

A student was diagnosed as having Wilson's disease only after the severe, intermittent inability to open his eyes led him to seek neurologic evaluation. Although lid-opening apraxia is usually a symptom of diffuse extrapyramidal disease, it has not previously been reported in Wilson's disease.

Nonutility of cineurograms in children with congenital heart disease.

Cineurography is often performed after cineangiocardiography to look for occult congenital urinary tract disease. The accuracy of cineurography was investigated in 171 patients by comparing cineurograms with renal sonograms. One hundred fifteen cineurograms (67%) showed both kidneys well enough to allow assessment of renal structure and function and the results were confirmed in 112 by ultrasonography; 3 cineurograms yielded false-positive results.

Acute complications of catheter therapy for congenital heart disease.

Although therapeutic catheter procedures for congenital heart disease are still developing, the number of procedures being performed allows statistically relevant review of the acute complications. Between January 1, 1984, and February 1, 1987, 417 such procedures were performed at The Children's Hospital, Boston; the age range of the patients was 1 day to 51 years (median 4 years, 6 months). Catheter procedures consisted predominantly of vascular dilations (peripheral pulmonic stenosis, 97; valvular pulmonic stenosis, 67; valvular aortic stenosis, 62; recurrent coarctation, 49) and embolizations (double umbrella device, 36; steel coils, 45).

Traumatic internuclear ophthalmoplegia.

Unilateral and bilateral internuclear ophthalmoplegia can rarely be caused by trauma. This article illustrates a traumatic pontine hemorrhage associated with bilateral internuclear ophthalmoplegia.

Balloon dilation angioplasty of postoperative aortic obstructions.

Balloon dilation angioplasty (BDA) was attempted 29 times in 27 patients, aged 3 months to 22 years, with postoperative aortic obstructions. Previous operations included end-to-end anastomosis (n = 10), subclavian flap angioplasty (n = 7) and patch angioplasty (n = 3) for aortic coarctation, end-to-end anastomosis for interrupted aortic arch type B (n = 4) and aortic arch reconstruction for hypoplastic left heart syndrome (n = 3). Two of the patients with interrupted arch had multiple areas of obstruction.

Balloon dilation of the aortic valve: studies in normal lambs and in children with aortic stenosis.

To evaluate the risks of and optimal method for valve dilation in aortic stenosis, balloons of different sizes were used to dilate the normal aortic root in 16 lambs and then stenotic valves in 15 children. In the lambs, inflated balloon to aortic anulus diameter ratios ranged from 0.9 to 1.

Upbeat nystagmus: clinicopathologic study of two patients.

Two patients had upward nystagmus on forward gaze before they died with acute caudal brainstem dysfunction. Bilateral dorsal paramedian damage in the rostral medulla, involving the perihypoglossal nuclei, was probably the critical lesion responsible for upbeat nystagmus.

Transcatheter umbrella closure of congenital heart defects.

Between October 1984 and September 1986, we attempted transcatheter umbrella closure, using the Rashkind PDA occluder, of 12 congenital or postoperative cardiovascular defects (other than patent ductus arteriosus [PDA]) in 11 patients. In each, we used the umbrella for closure because the defect was too short and/or too large to close with conventional transcatheter methods. The defects included three post-Glenn venous communications (superior vena cava-right atrium, n = 2; azygos vein to inferior vena cava), four congenital "interatrial defects" producing cyanosis ("coronary sinus" septal defect, left superior vena cava to left atrium, patent foramen ovale, left inferior vena cava to left atrium), and five non-PDA systemic-to-pulmonary arterial communications (two congenital and three postoperative).

Trochlear nerve pareses with brainstem lesions.

Three cases of radiographic localization of fourth nerve lesions are reported: case 1--bilateral pareses due to traumatic contusion; case 2--left paresis from a collicular gunshot wound; and case 3--bilateral pareses due to a (cysticercal) cyst in the caudal cerebral aqueduct.

Wrong-way deviation of the tongue with hysterical hemiparesis.

Strong deviation of the protruded tongue towards the "normal" side is an old sign of hysterical hemiparesis. The following four cases illustrate that hysterical tongue deviation continues to offer a valuable diagnostic clue.

Percutaneous balloon valvotomy of congenital pulmonary stenosis using oversized balloons.

Percutaneous balloon valvotomy was attempted in 27 patients (aged 6 days to 19 years, median 2 years, 11 months) with unoperated typical valvular pulmonary stenosis using a balloon 7 to 60% (mean 30%) larger than the valve anulus. One patient had undergone a previous balloon valvotomy elsewhere. To achieve an oversized dilation diameter in three larger patients, two balloons were inflated side by side.

Creation and maintenance of an adequate interatrial communication in left atrioventricular valve atresia or stenosis.

Patients with left atrioventricular (AV) valve atresia or stenosis were studied retrospectively to determine the incidence of early and late failures of procedures to enlarge an interatrial communication. The 61 patients underwent 80 procedures: 5 balloon atrial septostomies, 12 blade atrial septostomies and 63 surgical septectomies. No balloon septostomy provided adequate long-term palliation.

Long-term follow-up of valvotomy before 1968 for congenital aortic stenosis.

The clinical course of 59 patients who underwent valvotomy for aortic stenosis before 1968 was reviewed. All were older than 1 year at the time of operation. Mean follow-up period was 17.

Bilateral ocular motor signs after tentorial herniation in 25 patients.

Twenty-five patients with upper brain-stem damage after severe transtentorial herniation showed a variety of residual oculomotor signs. Pupillary abnormalities (19 patients), ptosis (15 patients), internuclear ophthalmoparesis (13 patients), vertical gaze paresis (14 patients), and third-nerve dysfunction (11 patients) were the principal findings.

Fibrinolytic therapy for femoral arterial thrombosis after cardiac catheterization in infants and children.

The charts of 79 patients who required femoral arterial (FA) thrombectomy after cardiac catheterization were reviewed. Fifteen patients (19%) had poor pulses after thrombectomy and 2 had an extremity amputated. One thousand consecutive patients undergoing cardiac catheterization were also studied to prospectively determine the safety and efficacy of systemic fibrinolytic therapy for treatment of FA thrombosis.

Percutaneous transvenous balloon valvotomy in a patient with severe calcific mitral stenosis.

Percutaneous transvenous balloon mitral valvotomy was performed successfully in a 57 year old man with refractory congestive heart failure due to calcific mitral stenosis. Cardiac surgery was not an option because of other major medical problems. Balloon mitral valvotomy was performed using the transseptal technique.

Percutaneous mitral valvuloplasty in an adult patient with calcific rheumatic mitral stenosis.

A 75 year old man with long-standing rheumatic mitral stenosis who refused surgical intervention was treated with percutaneous balloon valvuloplasty. Prevalvuloplasty evaluation revealed a heavily calcified mitral valve, a mean transvalvular gradient of 18 mm Hg, a Fick cardiac index of 1.7 liters/min per m2, a mitral valve area of 0.

Vertical diplopia.

At this point, it is customary for all hands to gather round the old decision tree and whittle on a few algorithms. Because I have been chastened by occasional painful falls from slippery decision branches, however, I must make do with a short reprise. Some nodal points in analyzing vertical diplopia include: (1) evidence of central nervous system involvement; (2) pupillary sparing; and (3) the presence of proptosis of orbital congestion.

Acute bilateral ophthalmoplegia: 60 cases.

Of 60 patients with acute bilateral ophthalmoparesis, the cause was within the brainstem in 18 (pretectal infarct, phenytoin toxicity, and Wernicke's syndrome), in cranial nerves in 26 (Guillain-Barré or Fisher syndrome, tuberculous meningitis), within the cavernous sinuses in 8 (tumors or infection), and at the myoneural junction in 8 (myasthenia or botulism).