Familial occurrence of discrete subaortic membrane.

The first case of multiple family members with discrete subaortic membrane and no other congenital defects is presented. One family member presents with findings suggesting a forme fruste of this disease. Increased surveillance of family members of individuals with discrete subaortic membrane is warranted, as the clinical findings of mild subaortic obstruction may be indistinguishable from those of an innocent flow murmur.

Pacemaker contact sensitivity: clinical recognition and management.

We report a patient in whom vesicular lesions of the skin developed overlying the pacemaker at intervals of 3 to 8 months after each of three consecutive insertions. Patch skin tests were positive for titanium and polyurethane sensitization. Although pacemaker contact sensitivity is rare, its recognition is of vital importance to the pacemaker-dependent patient.

Recurrent obstruction after subclavian flap repair of coarctation of the aorta in infants. Can it be predicted or prevented?

Recoarctation is a problem in some patients after subclavian flap aortoplasty. To investigate the reason for recoarctation, we reviewed the records of 26 infants who underwent subclavian flap repair for symptomatic coarctation of the aorta at less than 3 months of age between June, 1979, and December, 1983. Age at repair ranged from 2 to 65 days (median 16 days) and weight from 2.1 to 4.9 kg (median 3.4 kg). In 14 patients the coarctation was associated with significant intracardiac defects (complex in six). There were two intraoperative deaths and one early death (surgical mortality 12%). The survivors were followed from 6 weeks to 66 months (median 12 months). Five survivors (22%), all operated on at less than 14 days of age, developed severe recoarctation 6 weeks to 6 months (median 5 months) after repair. The obstruction appeared to be due to lumen obliteration by shelf-life posterior wall tissue. Morphometric analysis of preoperative angiograms showed no correlation between recoarctation and distance between the left subclavian artery and the site of coarctation, length of the isthmus, diameter of the isthmus, combined cross-sectional area of the left subclavian artery and isthmus, or the ratio of the combined cross-sectional area of the left subclavian artery and isthmus to the cross-sectional area of the descending thoracic aorta. Recoarctation did not correlate with weight at operation, but it correlated significantly with age at aortoplasty (p = 0.02). The results suggest that intrinsic abnormalities of the periductal aortic wall are responsible for recoarctation after subclavian flap aortoplasty. Particular attention to this abnormal tissue at repair may prevent early recurrence in young infants.

Aneurysm of main pulmonary artery in a neonate with airway obstruction and heart failure: long-term survival after pulmonary artery aneurysmectomy and patent ductus arteriosus ligation.

A neonate was seen with complete atelectasis of the left lung secondary to compression of the left main bronchus by a congenital aneurysmal main pulmonary artery. Operation consisted of pulmonary artery aneurysmectomy and ligation of an associated patent ductus arteriosus. Follow-up (3 years after operation) demonstrated complete resolution of the atelectasis and congestive heart failure.

Vasovagal syncope in children requiring pacemaker implantation.

Four children presented with episodic loss of consciousness. Two of the children were siblings. Neurologic causes were initially suspected in all but extensive evaluations and EEGs excluded seizures. ECGs in one patient demonstrated first- and second-degree AV block and first-degree AV block in another. The QT and QTc intervals were normal in all. Eyeball pressure in all patients produced profound bradycardia. All patients became asymptomatic after the implantation of pacemakers, although one died 15 months afterward from another cause.