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Biomedical subjects

P J Hofschire

Publications and source records attributed to P J Hofschire.

49 records · Page 3Linked to original sources
PubMed1981

Changing indications for pacemakers in children.

Permanent pacemakers were implanted in 50 children. Indications were symptomatic sinus node dysfunction in 34 (68%), surgical block in 9 (18%), and congenital block in 7 (14%). Twenty-three (68%) of the 34 children with sinus node dysfunction had undergone prior cardiac operations. Only 4 of the 50 patients (8%) had electrode problems after a mean pacing time of 29.5 months (range, 1 to 96 months). All 35 of the mercury-cell pulse generators used in 28 patients ceased to function after an average useful life of 20.8 months (range, 1 to 51 months). The lithium-powered units in the 45 survivors all show satisfactory pacing after 5 to 44 months (mean, 28.1 months). With improved pacemaker technology, longer survival after complex repairs, and better monitoring techniques, the indications for cardiac pacing in children have broadened. Surgical block now is an indication in only a small fraction of the pediatric pacemaker population. Sinus node dysfunction accounts for an ever-increasing majority of the pacemakers we currently implant in children.

Adolescent↗
PubMed1979

Critical aortic coarctation: patch aortoplasty in infants less than age 3 months.

Twenty-three infants less than age 3 months (mean age 31 days) underwent patch aortoplasty for relief of coarctation of the aorta. All had intractable congestive heart failure, despite aggressive medical therapy. Each infant had other cardiac anomalies, including patent ductus arteriosus (83 percent) and ventricular septal defect (74 percent). All patients underwent closure of the ductus arteriosus and patch angioplasty of the aorta to produce a luminal diameter of at least 16 mm. In addition, 9 of the 17 patients (53 percent) with a large shunt ventricular septal defect underwent pulmonary arterial banding. There was one hospital death 42 days after operation secondary to bowel perforation and sepsis. Hospitalization beyond 21 days postoperatively was always due to other unrepaired cardiac lesions. The three late deaths at 3, 9 and 18 months after operation were associated with additional major anomalies. Fourteen patients have had postoperative catheterization. No gradient was found across the site of coarctation repair, but one patient had a gradient between the left carotid and left subclavian arteries. Surgical repair of critical coarctation of the aorta in infants can safely be offered despite the presence of other cardiac anomalies.

Aging↗
PubMed1979

Late complications following surgical repair of total anomalous pulmonary venous return below the diaphragm.

Between May, 1975, and June, 1977, we surgically repaired an infracardiac total anomalous pulmonary venous return in 4 infants under deep hypothermic cardiac arrest. All patients had pulmonary hypertension and a patent ductus arteriosus. All survived operation and required positive end-expiratory pressure with mechanical ventilation. Late complications included patent ductus arteriosus not visualized at initial catheterization, breakdown of an oversewn atrial septal defect repair, and pulmonary venous obstruction despite an anastomosis diamter of 1.5 to 2.5 cm. At reoperation, there was scarring and contraction at the junction of the pulmonary veins and left atrium. One infant with these complications died post-operatively. In another patient, there was kinking of the left lower pulmonary vein at its juncture with the left atrium. In view of these complications we currently recommend the following: ligation of the ductus arteriosus regardless of catheterization findings; a right-sided approach to avoid pulmonary vein kinking; prosthetic patch closure of the atrial septal defect to avoid excessive tension on suture lines and to maintain maximum left atrial size; stellate anastomosis to provide the largest possible drainage channel; awareness that an unusually high positive end-expiratory pressure may be required postoperatively; and early recatheterization.

Cardiac Catheterization↗
PubMed1977

Postoperative complete heart block in 64 children treated with and without cardiac pacing.

Between 1957 and 1973, a total of 64 children had complete heart block during intracardiac surgery were treated by one of three methods: (1) No pacemaker: Six of the 13 patients in this group had reversion to sinus rhythm. Seven patients continued to have complete heart block, and five of these died of Stokes-Adams episodes. Two patients continue to have complete heart block without pacemaker insertion. (2) Temporary pacemaker: Seventeen of 25 patients had reversion to sinus rhythm and the temporary pacemaker was removed; the other 8 continued to have complete heart block. Five of the eight died--three of Stokes-Adams episodes and two at the time of cardiac reoperation. Three remain asymptomatic without placement of a permanent pacemaker. Six died of postoperative complications unrelated to heart block or pacemaker implantation. None of the other 20 experienced Stokes-Adams attacks. There was one late death in this group. Although there are problems in using permanent pacemakers and electrode systems, in these patients with operatively induced complete heart block their use was associated with a low mortality rate.

Adolescent↗
PubMed1977

Pulmonary vascular disease complicating the Blalock-Taussig anastomosis.

The pulmonary vascular bed was evaluated following a Blalock-Taussig shunt performed in 36 patients for decreased pulmonary blood flow. The time of follow-up ranged from one to 21 years after operation. No patients developed severe pulmonary changes in less than eight years after institution of the shunt. Ten of 20 patients having a shunt eight years or longer developed some degree of pulmonary vascular disease, mainly in the form of intimal fibrosis. This was severe in six of the 20 patients. As the duration of the shunt increased, so did the incidence of pulmonary vascular disease.

Adolescent↗
PubMed1976

Echocardiographic features of combined hypertrophic and membranous subvalvular aortic stenosis: a case report.

Combined hypertrophic and membranous subvalvular aortic stenosis was detected by echocardiography. The diagnosis was established by the discovery of severe asymmetric septal hypertrophy, a membrane in the left ventricular outflow tract, narrowing of the left ventricular outflow tract, as well as high frequency fluttering and premature systolic closure of the aortic valve leaflets.

Aortic Stenosis, Subvalvular↗
PubMed1976

Cardiac valvular anomalies in Fabry disease. Clinical, morphologic, and biochemical studies.

The cardiovascular abnormalities were investigated in two unrelated hemizygous males with Fabry disease who had clinical mitral insufficiency. Postmortem examination of their hearts revelaed anatomic, ultrastructural and biochemical abnormalities resulting from defective activity of the lysosomal enzyme, alpha-galactosidase A. The ultrastructural and biochemical studies demonstrated the marked accumulation of the major glycosphingolipd substrate, trihexosyl ceramide, in the lyosomes of all the cardiac tissues examined; the greatest concentrations were found in the mitral valve and elft ventricular myocardium. Intriguingly, digalactosyl ceramide, a glycosphingolipid substrate not detectable in normal lung, vessel or cardiac tissues, was found increased only in the lung and right heart tissues. Morphologic and chemical examination of cardiac and systemic vessels demonstrated accumulation of trihexosyl ceramide in lysosomes of the vascular endothelium. These studies demonstrate that the progressive accumulation of trihexosyl ceramide in the lysosomes of the cardiac structures and vascular system leads to the multiple cardiovascular manfiestation of Fabry disease.

Ceramides↗
PubMed1976

Anomalous double blood supply to the lung.

We report an unusual case in which an apparently normal upper lobe of the right lung was supplied by major systemic arterial and pulmonary arterial vessels. The anomalous artery arose from the descending aorta. Following interruption of this vessel, the machinery-like murmur previously present disappeared.

Child, Preschool↗
PubMed1975

Ruptured ductus arteriosus. Aneurysm in an infant. Report of a survivor.

The diagnosis of a large ductus arteriosus aneurysm was made in a 3 1/2-month-old infant by cardiac catheterization following massive hemoptysis. Prompt surgical correction accounted for the patient's survival. Differential diagnosis of an intrathoracic mass should include a ductus arteriosus aneurysm even in the newborn period.

Diagnosis, Differential↗