Biomedical subjects
Publications and source records attributed to L E Alday.
One of conjoined thoracopagus twins, with separate hearts and a common pericardial sack, presented with respiratory distress because of a persistent arterial duct causing congestive heart failure in the neonatal period. Surgical ligation of the duct was performed prior to subsequent separation, with an excellent outcome.
Explore the source record for details and available documents.
We report a 6-year-old girl with superoinferior ventricles, criss-cross atrioventricular (AV) relationships with solitus atria, a D-loop, L-transposition of the great arteries, subpulmonary stenosis, but without a ventricular septal defect (VSD). The diagnosis was made by echocardiographic examination and was strengthened by cardiac catheterization and angiocardiography. Balloon atrial septostomy in the neonatal period and a modified Blalock-Taussig shunt at the age of 16 months were performed to alleviate hypoxia. Following the shunt operation, the clinical condition of the patient deteriorated with progressive cardiac enlargement, congestive heart failure, and tricuspid regurgitation. We assume that the absence of a VSD contributed to this deterioration. Subsequent improvement was obtained with balloon enlargement of the atrial septal defect and a bidirectional Glenn anastomosis.
Explore the source record for details and available documents.
A three year old girl with severe congenital mitral stenosis was successfully treated by percutaneous balloon dilatation of the mitral valve. Cardiac catheterisation and cross sectional and Doppler echocardiography indicated that the orifice of the mitral valve had doubled in area. A small atrial septal defect was found at follow up cardiac catheterisation and angiography. Balloon dilatation of the mitral valve is a reasonable alternative to surgical treatment for typical congenital mitral stenosis even in young children.
In 12 infants and children with hypertrophic cardiomyopathy (HCM), seen during a 5-year period, the incidence of the idiopathic or primary form was similar to that secondary to systemic disease. Five of the six patients with secondary HCM are reported. Pompe's disease, Friedreich's ataxia, and Noonan's syndrome were seen in one each. There were two patients with lentiginosis. The diagnosis of HCM and of the metabolic disorder was made simultaneously in the patient with Pompe's disease. In two patients, one with Friedreich's ataxia and the other with lentiginosis, the diagnosis of HCM preceded the manifestations of the associated disorder. The other two patients were referred to rule out heart disease. The diagnosis of HCM in infancy and childhood should be considered with the possibility of being a secondary form, and patients with known associations with HCM should be referred for early diagnosis of heart conditions.
Explore the source record for details and available documents.
Cor pulmonale and severe congestive heart failure secondary to chronic upper airway obstruction developed in a three-year-old girl with congenital ankylosis of the temporomandibular joint complicated by frequent respiratory infections. Nearly absent mouth opening, micrognathia, and mandibular retroposition with resultant glossoptosia obstructed the airway. Medical treatment followed by a tracheostomy and bilateral condylectomy relieved the obstruction allowing normal function of the temporomandibular joint. The clinical, electrocardiographic, radiologic, and hemodynamic findings returned to normal. Congenital ankylosis of the temporomandibular joint has not been previously reported as a cause of cor pulmonale secondary to upper airway obstruction.
Total anomalous pulmonary venous connection (TAPVC) is failure of development of the common pulmonary vein, with consequent ersistence and enlargement of embryonic collaterals between the lungs and the systemic veins. In the great majority (91 of 93 autopsied cases, 97 per cent, no remnant of the common pulmonary vein was found, supporting the concept of agenesis or involution of the common pulmonary vein. Occasionally, a cordlike strand was found in the location of the common pulmonary vein (in three of 93 cases, 3 per cent), supporting the concept of atresia of the common pulmonry vein. The order of frequency of the various types of TAPVC was: left innominate vein ("snowman"), 26 perent cent; subdiaphragmatic, 24 per cent; coronary sinus, 18 per cent; right superior vena cava, 15 per cent; right atrium, 8 percent; mixed, 5 per cent; azygos, 2 per cent; and left supeior vena cava, 2 per cent...
Ventricular aneurysms in children are unusual. Three patients with cardiomyopathy associated with angiographically proved left ventricular aneurysms in this age group are reported. Two of them were girls. The ages were 20 months, 7 years, and 14 years. Heart failure was present in all patients. There was radiological evidence of cardiomegaly in all three, and the electrocardiogram showed signs of necrosis in two of them. Selective left ventricular angiography disclosed generalized hypokinesis in all patients. One child had an aneurysm of the diaphragmatic wall. In another the aneurysm was localized in the muscular ventricular septum, causing severe subpulmonary stenosis by encroaching in the right ventricular outflow tract during systole. The third patient had an aneurysm of the left ventricular free wall partly encircling the left ventricle. The coronary arteries appeared normal in all cases. The clinical features of the underlying disease were not altered by the presence of the aneurysm except in the patient with the septal aneurysm and subpulmonary stenosis. In this patient the aneurysm was successfully resected.
A case of a 48-year-old woman with frequent syncopal episodes is reported. The electrocardiogram showed high degree AV block with narrow QRS complexes. The His bundle electrogram displayed a split His deflection indicating impairment of conduction within the His bundle of the Mobitz II type. The AH interval was prolonged and Wenckebach phenomenon occurred at the same atrial pacing rate before and after atropine administration. During spontaneous or induced high grade AV block an escape rhythm originating in the distal His bundle was observed. A secondary study performed one year later showed progression to complete AV block. Both His potentials were present, one following the atrial and the other preceding the ventricular deflection. The H'V interval was prolonged and a further lengthening was seen after ajmaline. All these findings indicated proximal, mid, and distal disease of the His trunk.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Woolly hair and palmoplantar hyperkeratosis is an infrequent autosomal recessive ectodermal disorder that may be associated with arrhythmogenic right ventricular dysplasia (Naxos disease) or dilated cardiomyopathy. We report the sporadic occurrence of the aforementioned skin-hair anomalies in a patient with physical findings compatible with Noonan's syndrome and hypertrophic cardiomyopathy-an association heretofore not described.